["430 15.2 Aging Health and Performance Changes DISABILITY COMMON REL ATED HEALTH PREVENTION STR ATEGIE CONDITIONS Cerebral Palsy Pain Routine exercise Fatigue Monitor and query routinely Work simplification Ergonomic evaluations Energy conservation Musculoskeletal Monitor and query routinely Contractures Joint protection strategies Hip pathology Routine exercise Knee pathology Biomechanic and ergonomic a Foot or ankle pain Back pain Routine exercise Calcium\/vitamin D supplemen Osteoporosis\/fractures Fracture and fall prevention; e Routine monitoring Neurologic Adjust medications with repor Spasticity Query for changes; high index Seizures Spinal stenosis Monitor and query routinely Nerve entrapments Urinary conditions Incontinence UTIs Respiratory conditions Routine monitoring Infection Immunization Sleep apnea Query sleep hygiene Monitor and query routinely; re Gastrointestinal Nutritional management Constipation GERD Routine exercise Obstruction Education and prevention Deconditioning Falls","ES TREATMENT STR ATEGIES assessments Exercise prescription nt education Query\/evaluate sleep; manage as needed rted change Evaluate for pain etiology and treat x of suspicion for pathology Modify equipment or workplace Evaluate mental health and manage ecognition of severity Progress to pain management program Focal musculoskeletal evaluation Tone management Modify equipment. workplace, biomechanics of function Physical therapy prescription Adjust orthoses DEX A evaluation Consider treatment when multiple fractures Exercise when appropriate Tone management; medications, BTX injections, ITB Seizure management Radiologic evaluation Electrodiagnosis Surgical referral when appropriate Urodynamic evaluation Scans\/radiographs Medications and CIC when needed Urology referral as appropriate Scoliosis evaluation Sleep study and management Specialty referral as needed Adjustment to bowel program regimen Specialty referral when appropriate Therapy prescription; focus on strength and aerobics Reconsideration of equipment","Mental health Routine monitoring Sexual functioning Query of support, living arrang Spina Bifida Health maintenance Provide with education; approp Urologic\/renal disease function UTIs Assist with environmental mod Incontinence assessments as able Vesico-ureteral reflux End-stage renal disease Assure pregnancy high risk ne Bladder cancer Monitoring (see Table15. 4) Routine monitoring; UTI freque Musculoskeletal Shoulder pain\/overuse urodynamics Scoliosis Maintain routine urology appo Joint pain Routine exercise, especially st Osteoporosis\/Fracture shoulder Neurologic Monitor and query routinely Hydrocephalus Joint protection strategies Chiari malformation Routine exercise Tethered cord Biomechanic and ergonomic a Epilepsy Calcium\/vitamin D supplemen Obesity Education and fall prevention Routine monitoring Pressure ulcers Query for changes Maintain neurosurgery appoin Routine neurology appointmen Monitor weight Routine exercise Nutrition management Frequent position change Monitor skin, nutrition, equipm 431","gements Specialty referral as appropriate priate modality for level of Referral for psychological and social support dification for routine Use of community resources eeds are met Following pregnancy, support may be needed in the home ency, renal scans, Appropriate management, consideration of alternatives for ointments treatment With change consider neurologic evaluation as cause trengthening posterior Focal musculoskeletal evaluation assessments Evaluate for neurologic change with new symptoms nt Modify equipment (possible power wheelchair), workplace, ntments nts with active epilepsy biomechanics of function Therapy prescription ment, change in function Neurosurgical evaluation Post-surgery, may require rehabilitation admission Cognitive and functional assessments post-intercurrent events to assure safe community living Nutrition referral Exercise prescription Modify positioning or pressure relief equipment Assure good nutrition Appropriate care for ulcer staging May need change to tone management Surgical referral Continued","432 15.2 Aging Health and Performance Changes (Continued) DISABILITY COMMON REL ATED HEALTH PREVENTION STR ATEGIE CONDITIONS Pulmonary restriction Monitor for infection Pulmonary infection Immunizations Bowel incontinence Monitor and adjust program fo Lymphedema Monitor, use of compression a Latex allergy Limit exposure to latex Mental health Monitor routinely Sexual functioning Provide with education; approp Spinal cord Health Maintenance function injury Urologic\/renal disease Assist with environmental mod assessments as able UTIs Renal calculi Urology referral for fertility\/per Incontinence Assure pregnancy high risk ne Reflux Monitoring (see Table 15.4) Routine monitoring; UTI freque Musculoskeletal urodynamics Shoulder pain\/overuse Maintain routine urology appo Scoliosis Routine exercise, especially st Other pain complaints shoulder Osteoporosis\/fracture Monitor and query routinely Joint protection strategies Routine exercise Biomechanic and ergonomic a Calcium\/vitamin D supplemen Education and fall prevention","ES TREATMENT STR ATEGIES or change Evaluate for neurologic change with new symptoms and elevation at first sign Consider sleep study or O supplement priate modality for level of 2 dification for routine r formance Evaluate for neurologic change with new symptoms eeds are met Referral for lymphedema program and prescribed ency, renal scans, ointments compression garments Modify equipment if needed trengthening posterior Acute event treatment Appropriate recognition in medical record, personal assessments nt acknowledgement Specialty referral as appropriate Referral for psychological and social support Use of community resources Following pregnancy, support may be needed in the home Appropriate management, consideration of alternatives for treatment With change consider neurologic evaluation as cause Focal musculoskeletal evaluation Evaluate for neurologic change with new symptoms Modify equipment (possible power wheelchair), workplace, biomechanics of function Therapy prescription Adjust orthoses, footwear","Neurologic Routine monitoring Spasticity Query for changes Autonomic dysreflexia Adjust medications with repor Pulmonary conditions Monitor for infection Ventilator dependency Immunizations Consider diaphragm or phrenic Pressure ulcers Frequent position change Monitor skin, equipment, chan Bowel incontinence Latex allergy Monitor and adjust program fo Limit exposure to latex Mental health Monitor routinely Sexual functioning Provide with education; approp Limb deficiency Health Maintenance function Overweight or obesity Assist with environmental mod Pain assessments as able Deconditioning Urology referral for fertility\/per Falls Assure pregnancy high risk ne Monitoring (see Table 15.4) Monitor weight and nutrition Routine exercise Routine exercise Monitor and query routinely Work simplification Ergonomic evaluations Energy conservation Education and falls prevention Routine exercise 433","rted change Tone management; progress to more aggressive strategies c nerve pacing Evaluate for neurologic change, painful symptoms, bowel\/ nge in function or change bladder etiologies, fractures, pressure ulcers with more frequent AD symptoms priate modality for level of Evaluate for neurologic change with new symptoms dification for routine Consider sleep study or O2 supplement r formance eeds are met Modify positioning or pressure relief equipment Appropriate care for ulcer staging n May need change to tone management Assure good nutrition Surgical referral as appropriate Evaluate for neurologic change with new symptoms Acute event treatment Appropriate recognition in medical record, personal acknowledgement Specialty referral as appropriate Referral for psychological and social support Use of community resources Following pregnancy, support may be needed in the home Exercise or therapy prescription Referral to nutritionist if indicated Modify prosthesis as needed Focal examination and evaluate\/treat Exercise prescription Modify equipment or workplace Progress to pain management program Adjust prosthesis as needed Therapy prescription; focus on strength and aerobics Adjust prosthesis as needed Consider other equipment Continued","434 15.2 Aging Health and Performance Changes (Continued) DISABILITY COMMON REL ATED HEALTH PREVENTION STR ATEGIE CONDITIONS Intellectual CVD\/PVD Reduce risks disability Routine exercise Health Maintenance Monitor as indicated CVD Obesity Monitoring (see Table 15.4) Respiratory disorders Routine monitoring Epilepsy Routine exercise Nutrition management Routine monitoring Immunizations Routine Neurology appointmen Osteoporosis\/fractures Query and monitor Poor oral health Routine exercise Mental health Calcium\/vitamin D supplemen Sexual functioning Fracture and fall prevention; e Monitoring Health maintenance Assist with environmental acc Routine monitoring Reduce life events Query of support, living arrang Provide with education; approp function Assist with environmental mod assessments as able Assure pregnancy high risk ne Monitoring (see Table 15.4)","ES TREATMENT STR ATEGIES Referral and management as indicated nts Exercise prescription nt Referral for nutritional consultation education Consideration of sleep apnea, need for O2 supplement cessibility if able Assist with change in community living arrangement as gements needed priate modality for level of DEX A evaluation dification for routine Consider treatment when multiple fractures eeds are met Exercise when appropriate Specialty referral as appropriate Referral for needed supports Use of community resources Following pregnancy, support may be needed in the home","Down Mental health Monitor syndrome Alzheimer dementia Reduce life events such as mo Depression Routine monitoring Endocrine Annual TSH monitoring Hypo- or hyperthyroid Diabetes Reduce vascular risk Routine exercise CVD Monitor Mitral valve prolapse Monitor Monitor Celiac disease Query and monitor Hearing loss Calcium and vitamin D Sleep apnea Routine exercise Musculoskeletal Monitor for urologic change, d A r thritis weakness, bowel changes, p Osteoporosis Atlantoaxial instability Obesity Routine exercise Respiratory infections Nutrition management Sexual functioning Monitor Immunizations Health maintenance Provide with education; approp function Assist with environmental mod assessments as able Monitoring (see Table 15.4) DE X A, dual energy X-ray absorptiometry; BT X, botulinum toxin; ITB, intrathecal baclofen; UTI, urinary tract inf dysreflexia; CVD\/AVD, cardiovascular disease\/atherosclerotic vascular disease; TSH, thyroid-stimulating horm 435","oves Behavior management dysphagia, spasticity, Medications as needed pain Specialty referral as appropriate Referral for needed supports priate modality for level of Use of community resources dification for routine Medication management Diet management Evaluation, treatment per study Management per gastroenterologist Consider amplification if appropriate Sleep study and management Evaluate pain complaints appropriately Medications as appropriate Therapy prescription Consider treatment if multiple fractures Full evaluation of performance changes; radiographs and referral as appropriate Referral to neurosurgery with acute loss Referral for exercise program Referral for diet management Medications, possible O supplement 2 fection; CIC, clean intermittent catheterization; GERD, gastroesophageal reflux disease; AD, autonomic mone.","436 Pediatric Rehabilitation Through a large database in California defined by can be comparable to that of the community at large financial and service support needed and especially (16). In a population-based study of adults with cere- representative of the more severely impaired individu- bral palsy in a mid-sized metropolitan area, persons als with CP, survival of higher-functioning adults was with cerebral palsy were generally healthy (based on close to that of the general population (26). Strauss et al clinical information and self-report), but noted wor- also reported with this same database that older sub- ries and concerns about their health status and futures jects who had lost the ability to walk by age 60 years (34). Self-perceived health ratings and life satisfac- had poorer survival and that those who had the most tion may be related to the presence of pain or func- severe disabilities rarely survived to age 60 years (27). tional changes over time, but not to the severity of A later report by Strauss et al noted improved survival impairment (35\u201337). Despite reports of good health, a for adults with gastrostomy tubes in particular over a Canadian publication notes adults with CP attended 20-year period (28), indicating improvements in treat- outpatient physician visits 1.9 times higher than age- ment and care of the most fragile individuals with high matched peers (38). levels of impairment. Additional information from this database, weighted towards a more severely impaired The functional status of adults with CP is not cohort, reports standardized mortality ratios, noting a static over time, and with aging there can be modest higher mortality in general at 8.4, and as high as 13.8 decreasing function, as there is for the general popu- in the most severe group (29). There was a decrease lation. A number of studies, both in the United States in this discrepancy with age, which may indicate a and abroad, with small to large convenient samples, healthy survivor effect and increasing mortality in the have noted that about a third of subjects report modest general population. Respiratory etiologies as cause-of- to significant decreases in walking or self-care tasks death standard mortality ratio was 15, which is lower (16,27,39\u201341). Changes in dressing and walking with than is generally thought, and the highest overall for relative sparing of other self-care or social activities all ages was intestinal obstruction. were reported in two of these studies (16,27). Day et al used the large California database to determine the Reports from abroad also identify life expectan- probabilities of loss or gain of walking skills into adult- cies for adults with CP to be close to the general pop- hood for those with CP (42). They noted that by age ulation for those with mild to moderate impairments. 25 years, there would unlikely be any improvement The Western Australia Cerebral Palsy Registry noted in walking skill and most would not change over the the strongest single predictor of mortality was intel- next 15 years, although there could be some decline. lectual disability, with survival exceeding 92% for IQ\/ Therefore, the reason for even modest decreasing skill DQ scores >34 (30). This study noted motor impair- is not clear and may be related to progressive neuro- ment severity increased the risk of early mortal- logic problems (eg, cervical spine stenosis, radiculop- ity, with mortality declining after age 5 to 15 years, athy), lack of environmental modifications, pain, no and remaining steady at 0.35% for the next 20 years. access to or participation in exercise or activity pro- Providing insights on era of disability onset, Hemming grams, aging, or other medical conditions. et al reported on adults with CP in the 1940\u20131950 birth cohort in the UK. Assuming survival to age 20 years, Decreased independence (increased need for assis- almost 85% survived to age 50 years compared to 96% tance) in mobility and self-care is a common complaint of the general population (31). Again comparing to the of adults with mobility impairments. The reasons for general population, many of the deaths noted in ages change are varied, and may include those related to 20s\u201330s were respiratory, and deaths in ages 40s\u201350s age changes (eg, decreased endurance, flexibility, were circulatory conditions and neoplasms. Few deaths strength, or balance), progressive pathology or second- in adulthood were attributed to CP, although the ner- ary conditions (eg, pain, contractures, spasticity, oste- vous system was implicated more than in the general oporosis and fractures, stenosis), or personal choices population. The notion of increased neoplasm as cause (eg, use of powered mobility to conserve energy). The for death rates is echoed by the large California data- change in mobility is often a response to a second- base noting a three-times-higher rate for breast cancer ary condition or age-related change. Falls may also be in CP than in the general population, and this may such a response. Significant change in mobility or falls be related to severity as well as poor screening (29). should not automatically be accepted as a part of a Survival rates for children of today may not necessar- congenital or childhood-onset disabling condition in ily be extrapolated from any of these studies. adult years; treatable etiologies should be sought. Health and Functional Status It has been suggested through cross-sectional and convenience samples that adults with congenital The general health of adults with CP is self-reported or childhood-onset disabilities may show musculo- as good or satisfactory to excellent (32,33), and this skeletal or performance changes typical of advanced aging earlier than their nondisabled peers (32,26,43). These observations require confirmation through","Chapter 15 Aging With Pediatric Onset Disability and Diseases 437 longitudinal controlled studies. While risk factors self-manage their pain complaints (54), and for those may predispose a person to these changes, they are, who seek medical care, report is minimal improve- as yet, unproven. If these earlier-than-expected aging ment and few options offered (55). changes are confirmed, they should be considered sec- ondary conditions. Musculoskeletal and Neurologic Conditions Pain and Fatigue Contractures. Contractures are a common secondary condition, and reported in multiple case series. Their Pain is the most consistent health condition reported impact on functional status or general health care by adults with cerebral palsy (17,32,44,45). It has been needs is variable. Increasing contractures, particularly reported in a number of samples of adults with CP when associated with pain or increased spasticity, at a variety of ages to be 30% to 80%, with activity may be an indication of progressing pathology. Aging limitation from this at >50%. For this reason, it will changes include decreased flexibility, and the clini- be covered as a separate topic. Pain may be present cian must distinguish pathological causes of increas- for a variety of reasons; it may be acute, recurrent, or ing contracture through appropriate diagnosis. chronic. Increased spasticity, weakness, falls, or pro- gression of contractures or deformities can result from Osteoarthritis. Because of the significant pain com- pain, particularly when pain is not reported because of plaints that adults with CP offer, it is often stated that communication difficulties or severe intellectual dis- there is an early onset of osteoarthritis. Conceptually, ability. Because of the high prevalence, the health care this has been explained by unusual and possibly provider should try to elicit complaints or indications increased forces on joints that may have malalign- of pain, and evaluation, diagnosis, and intervention ment and\/or deformity, and associated with under- should ensue. Pain is often the reason for a change in lying weakness and poor motor control (32). In fact, function, living arrangement, or social interaction. health care providers often will make a presumed diagnosis of \u201carthritis\u201d for pain complaints in adults Pain is usually identified by proximity to a joint, with disabilities. Clinically, it is not surprising to and less often a limb. Most people report \u201carthritis\u201d as find significant arthritic changes with radiographs the etiology of these pain complaints; however, these of painful joints, and sometimes at young adult pains may originate from either joints or muscles. A ages. However, the presence of early-onset arthritic good history and clinical exam will help sort out the changes has been documented by case reports, and issues and direct appropriate treatment. Back, leg, studies that report arthritis among subjects base this and hip pain complaints are common in persons with information on self-report of arthritis or presence of cerebral palsy (46,47). There are usually more pain pain. Often, the pain complaint is not evaluated fully, complaints in those with spasticity (46). It has been and may have an etiology in soft tissue injuries or reported that fatigue often incites pain, and exercise problems and not degenerative changes within the most commonly relieves pain (46,48). joint. There may, in fact, be premature osteoarthri- tis, but it has not been documented definitively. Of Fatigue is a common complaint of adults with CP, importance is the recognition of pain, appropriate and is associated with pain (49). It is also associated with evaluation, and treatment. deterioration of skills and low life satisfaction, with no association with any specific type or severity of CP. As Hip Pathology. Degenerative changes have been noted noted, it may incite pain. The fatigue may also be associ- radiographically in dislocated and subluxed hips, not ated with the reported coping strategies sometimes used always related to weight bearing activities, in persons for chronic pain by adults with CP (50). Sleep disruption with cerebral palsy (44,56). Use of tone reduction strat- should also be questioned since it is commonly seen with egies may be helpful. Femoral head resection as a treat- pain and fatigue. Anecdotally, the pain\/fatigue complex ment strategy for control of pain in hip disease for persons appears to respond positively to directed pain manage- with cerebral palsy has been suggested; however, pain ment, good sleep hygiene, medications, and exercise. often persists or recurs postoperatively (57\u201360). Total hip and knee replacements as a treatment option for Appropriate management includes early identifi- pain from severe arthritis in adults with cerebral palsy cation of the problem and its source. Common mus- are becoming more common; however, as their lifelong culoskeletal etiologies include poor ergonomics and efficacy remains unknown (61\u201364), revision may be biomechanics in tasks (secondary to deformity or anticipated with placement at younger ages. limited motor control (41)), underlying weakness and therefore overuse (51), hypertonia (52), and degenera- Knee Pathology. Knee contractures are common in those tive joint disease (53). Typical management strategies who do not walk and in those who walk with obvious should be offered, and referral for additional inter- ventional, orthopedic, or neurosurgical consultation should be considered. However, adults with CP tend to","438 Pediatric Rehabilitation knee flexion and crouch. Not all knee contractures are neck mobility. When no surgical intervention is under- painful. Tone management may improve range, func- taken, a frank discussion of possible respiratory com- tion, and pain. Patella alta may develop over time, promise and the future need for ventilator assistance and pain or chondromalacia may result. Joint laxity should be provided. may also be present. Modalities, exercise, kinesiotap- ing, and other interventions may be helpful. There Peripheral Neurologic Compression. Radiculopathies may are advocates for patellar tendon advancement surger- be a cause for painful complaints, and appropriate eval- ies, with or without distal femoral extension osteoto- uation and treatment should ensue. It is most impor- mies, in adolescents and young adults to improve pain tant that treatment strategies are based on the person\u2019s and restore knee function in gait, confirmed on gait history of function, that there is effective input from analysis (65). that person or their care provider, and that practical outcome goals are identified. Although not as common Foot or Ankle Pain. Again from biomechanical factors, as a musculoskeletal etiology, nerve entrapment is also contractures and pain may develop. Typical interven- a cause of pain. The most common nerves and areas of tions may assist including orthoses, but not all bracing entrapment as reported by adults with CP are the same or shoe inserts are helpful, and biomechanics must be as those susceptible to compression in the nondisabled taken into account. Plantar fasciitis with appropriate population: the median nerve at the carpal tunnel and treatment should be considered. the ulnar nerve in the hand distally and at the elbow. Compression points are often related to use of crutches, Spine Pathology. In people with cerebral palsy, severe transfer techniques, propelling wheelchairs, or exist- motor impairment is associated with scoliosis and ing deformity. Work-related or positional activities may other deformities (66). Scoliosis may progress dur- also cause entrapments, just as in the nondisabled pop- ing adulthood, and those at 50 degrees or greater at ulation. There is no reported increased incidence in skeletal maturity may deteriorate more rapidly (67). CP. All hand pain or sensation change does not repre- Scoliosis can cause seating and pressure problems, sent nerve entrapment. Often, these complaints are impaired respiratory function, and pain (52,67,68), actually problems of repetitive motion or are position- and may be associated with windswept hips and pres- related. While they may be ascribed to carpal tunnel sure sores (52). It has been reported that spinal fusion syndrome, they often respond poorly to surgery (77). improves the quality of life for those with CP (69). Appropriate testing (including electrodiagnostic test- ing) is necessary to determine their etiology. Where Spinal stenosis must be ruled out whenever sig- treatment options are similar for disabled and nondis- nificant functional change is noted, particularly for abled adults, some modification of management will change in or loss of walking skills, increased leg be required if functional independence is changed by spasticity, change in bladder habits, neck pain, vague or during treatment. sensory changes, and (late) change in arm and hand function (70\u201372). A tethering effect on the spinal cord Osteoporosis. Osteoporosis has been documented in at also may occur, resulting in cranial nerve changes. least 50% of children and adults with cerebral palsy Some early reports noted a higher risk in those with (78,79). The aging process may exacerbate this issue, an athetoid or dyskinetic component (73,74); however, as does anticonvulsant use and mobility impairment. more recent reports show these problems are present Pathologic fractures occur typically in the long bones, in spastic forms of cerebral palsy as well. While it is but frequency data vary and no large studies of peo- generally held that stenosis is due to early spondylosis ple with cerebral palsy have been reported. Low serum and compression, there may also be a predisposition 25-OH vitamin D concentrations are not identified to it in those with a congenitally narrow canal, espe- as a cause in most cases described in the literature cially at C4\u2013C5 (70,73). Diagnosis is made through (79). Typical screening devices, such as the Simple imaging studies, while comparative evoked poten- Calculated Osteoporosis Risk Estimation (SCORE), do tials may also be helpful in determining neurologic not accurately identify osteoporosis risk in women with function. Surgical decompression may prevent fur- disabilities (80); therefore, bone mineral density testing ther, often catastrophic, loss of function, but does not and counseling on fall risk is important for both women assure return of lost function, particularly in cases of and men with disabilities. Dual energy x-ray absorption longstanding compression with spinal cord atrophy. (DEXA) scans must be read with caution, since contrac- Recurrence at levels above or below surgical correc- tures often skew results. Recommendation is to use the tion may be noted (75,76). Postoperative management scan results of the distal femur, as is used in children planning should accommodate changes in functional with CP and contractures (81). Use of bisphosphonates capabilities and care needs. The presence of an athe- is described, but the functional improvement derived toid movement component will affect postoperative from these drugs over the long term is unknown. spine stabilization and possibly head positioning and","Chapter 15 Aging With Pediatric Onset Disability and Diseases 439 Additional Health Conditions was noted earlier. Use of vaccinations may be helpful, along with vigilance and monitoring. Respiratory prob- There are no comorbidities known to be associated lems may increase with progressive scoliosis, and aspi- with CP. As noted, general health is good. A recent ration from gastroesophageal reflux disease (GERD) or study of adults living in group homes from upstate dysphagia must be recognized. Sleep disorders related New York notes increasing health conditions with age to pulmonary problems should be considered with pro- for adults with CP as would be expected: cardiovascu- gressive scoliosis, especially with complaints of poor lar, respiratory, and hearing\/vision (82); this has been sleep, morning headache, or daytime sleepiness. replicated in Taiwan and Israel (83,84). Of interest is that in comparison to U.S. national norms, there are There has been suggestion that obesity is a prob- fewer cardiovascular risk factors than seen in the gen- lem in CP, and yet there are no studies to support this. eral population; either this is a healthier population or In fact, a small study of adults with CP identified mean there has not been effective screening and monitoring. body fat percentages and body mass indexes were In looking more critically at this population, the sever- within normal range, although 40% had heights below ity of the CP was related to increasing health problems the fifth percentile for age and gender. Fifty-five per- with aging more than the diagnosis of CP alone (85). cent reported dysphagia (88). Vision and hearing problems may have been present early, and as anticipated, there is an increase in vision Sexual Functioning and hearing problems with age (82). Women\u2019s sexual health and functioning is better Dental issues are reported for adults with CP (46). described than men\u2019s. Women with CP typically have Medications, nutrition problems, poor dental hygiene, limited participation in health maintenance activities and difficulty with access to dental care all contribute such as routine pelvic examinations, Pap smears, and to the ongoing problems into adulthood. breast examinations (33,89). Office visit planning is required for those with significant motor impairments Previously known associated conditions will per- to assure a complete examination. Attitudinal barriers sist into adulthood. Dysphagia will continue, and of health care providers often limit services and edu- monitoring is required. Constipation also persists, cation. However, women with CP are typically able to and adjustments to bowel programs may be needed. conceive and carry pregnancies to term without the Gastroesophageal reflux is often reported, but has not expectation of major complications related to their CP. been present at increased rates. Intestinal obstruction Use of contraceptives has not been well studied, and is reportedly common in CP, and in an upstate New consideration of thrombotic effects must be considered York cohort living in group homes, adults with CP had in choice of options. A commonly offered contraception an increased rate compared to other adults with devel- is nonestrogenic formulations such as Depo-Provera, opmental disabilities (85). although long-term effects are not well defined (87,90). Women with CP report fewer sexual encounters as Urinary incontinence may also continue, and compared to other women with disabilities (17,91). assurance must be made that there is no dyssyner- Women with early-onset disabilities also experi- gia or overflow with retention. Rosasco et al reported ence high levels of sexual desire compared to other adults with CP had a higher incidence of urinary tract women with disabilities, postulated as being related infections (UTIs) that was related more to severity to reduced social opportunities, frustrated satisfaction than the presence of CP (85), compared to other adults of sexual urges, discouragement of childhood sexual with developmental disabilities living in group homes expression, or perceived social stereotypes (91). in upstate New York. Neurogenic bladders in adults with cerebral palsy are only infrequently associated Men with CP also should receive information on with upper tract pathology (86). Some women report sexual functioning, including information on contra- that incontinence consistently occurs at a particular ception and protection. There have been no reported point of their menstrual cycle, often associated with problems with sexual functioning or fertility. increased spasticity (87). Urinary incontinence can be effectively addressed through well-established diag- Spinal Cord Dysfunction nostic and intervention approaches. There are no avail- able data that assess the adverse impact of urinary Spina bifida (SB) and spinal cord injuries (SCIs) are incontinence on social integration in cerebral palsy, the most common etiologies for spinal cord dysfunc- but anecdotal support for this association is abundant. tion (SCD) in childhood, although infectious, rheuma- In both men and women, urinary incontinence should tologic, demyelinating, and tumor etiologies are also be identified and addressed, regardless of age or other seen. The incidence and prevalence of SCD in general conditions. is low in a pediatric population. Earlier chapters have identified the decreasing incidence of both SCI and SB. Respiratory problems have been implicated as cause of death early in life and in early adulthood, as","440 Pediatric Rehabilitation The prevalence for both, and for SCD in general, are who walked during their teen years continued walk- only estimates, and are well below estimates for intel- ing as adults. lectual disabilities (ID) and CP. It is also estimated that life expectancy is increasing, and therefore, it is impor- The associated cognitive effects of SB influence tant to understand the lifelong health and functional the level of functional independence in adults. A small issues of adults with childhood-onset SCD. SCD usu- cohort report showed that most young adults with ally involves multiple organ systems at a high level; hydrocephalus and lesions at L2 or above were depen- these medical conditions are fairly well described; and dent for sphincter control, locomotion, and self-care, consequently, there may be more medical monitoring with an additional number requiring assist with trans- than in other conditions. There is significant overlap in fers and social interaction and communication (96). the long-term management of those with SCI and SB, Those without hydrocephalus or with hydrocephalus although there are disability-specific health issues and and lesions below L2 required assist with sphincter risks. This section will highlight what is known about control only. An additional small study reported more the health of adults with childhood-onset SCI and SB difficulties in independence and quality of life, with independently. For both subsets, adults are present- increasing numbers of shunt revisions (97). ing with health challenges, such as renal dysfunction, musculoskeletal problems, neurologic complications, Perceived health for a group of young adults in pulmonary conditions, pressure ulcers, and sexuality the Netherlands was related to physical functioning, and reproduction issues (see Table 15.2). as would be expected using a tool standardized for the general population, not for disability (98). Of interest Spina Bifida was that the domains associated with emotional health did not differ from the population group. Using the Life Mortality Satisfaction Questionnaire, again in the Netherlands, highest proportion of dissatisfaction was with finan- As noted, in general, both early and late survival has cial situation, partnership relations, and sex life, and improved over the past 20 years. There are few data- those with hydrocephalus were less satisfied with self- bases that maintain statistics for specific disability care ability and partnership relationships than those diagnosis groups, but there are databases that involve without hydrocephalus (99). Overall, the presence of specific sites of care for programs serving people with SB does not appear to be an important determinant of SCD. Today, children born with an open SB have at life satisfaction. least a 75% chance of living into early adulthood. There is a high correlation of childhood death with Urology\/Nephrology hindbrain dysfunction and posterior cervical decom- pression, requiring tracheostomies and gastrostomies Urinary and renal issues are common health problems (92). Common causes of death in adulthood are renal for those with SCD. Renal damage and renal failure failure and causes related to the central nervous sys- are among the most severe complications in SB (100), tem (CNS), with continued hindbrain dysfunction and and contributes to early and late mortality. unrecognized shunt malfunction (92,93). In general, typical strategies for management of Functional Status and Mobility neurogenic bladders are used with goals of prevent- ing UTIs, preventing renal calculi, managing detrusor There are no large studies to identify change in func- pressures to prevent upper tract problems, monitoring tion over time. Most studies identify mobility based renal function to prevent renal failure, and assuring on defect level without regard for American Spinal continence. Clean intermittent catheterization (CIC) Injury Association (ASIA) levels or declaration of com- is an effective long-term management strategy for plete or incomplete function. Lower lesions are asso- properly selected persons with neurogenic bladders ciated with higher walking abilities, with or without from SB (100,101), usually concomitant with medica- aids (92,94). A small cohort of adults with sacral-level tions. However, there is no consensus for the evalu- myelomeningocele was noted to have maintained their ation, follow-along studies, and general management walking abilities for low-sacral lesions, and almost or management of bacteriuria among SB programs 90% maintained walking in the high-sacral group responding to a national U.S. survey (102), and there (95). Complications reported included scoliosis, oste- is no data about long-term outcomes. Renal function, omyelitis, amputations, and spinal tethering. A single as measured by creatinine (Cr) clearance, intravenous small study in the Midwest identified that across the pyelogram (IVP), ultrasound, or scan, has been found spectrum of SB, mobility decreased from early child- to be normal in 47.7% of patients with SB and abnor- hood to early teen years (92). At least three-fourths mal in 46.1% (94). In patients with lumbar-level SB who undergo CIC and are dry between catheteriza- tions, only 38% have normal renal ultrasound and Cr clearance greater than 1.5mg\/dL (103). This correlates","Chapter 15 Aging With Pediatric Onset Disability and Diseases 441 well with the fact that renal failure is the leading cause (115), and mortality and morbidities into adulthood of death among patients with SB despite proper man- are not well characterized. agement and follow-up (93). Bladder cancer has been reported in adults with For adults with SB, almost 60% of hospital admis- long-term SCI, and it has also been reported in adults sions are for urologic reasons, with neurologic prob- with spina bifida (116). The characteristics appear to lems accounting for almost 21%, and dermatologic differ from adult-onset SCI patients with younger age problems almost 20%. Of the urologic admissions, onset, variable tumor histology and advanced stage, and almost half of these were for conditions such as UTI and poor survival. A case report cautions about recogniz- renal calculi (104). In one study, urinary tract stones ing pseudotumors of the bladder in SB and SCI (117). were responsible for about 30% of all renal compli- cations (93). Repeated UTI, along with pyelonephritis Urinary incontinence in adults with childhood- and an already compromised kidney, can lead to acute onset SCD can persist into adulthood, and can be a renal failure with loss of nephrons. Unfortunately, socially limiting condition. Up to 80% of adults with SB by the time the serum creatinine begins to rise, the can achieve social urinary continence (44), although patient will have already lost up to two-thirds of their a survey of persons identified in a state registry as nephrons (105). It is important to note serum Cr is having spina bifida reported only a slight majority of dependent on muscle mass, so in adults with SCD who the adults had achieved independence in urinary man- often have low muscle mass, the serum Cr may not be agement (118). For adults with SB, incontinence has indicative of the true renal function (106). been shown to be associated with partial employment or unemployment (119). There has been an attempt to A study comparing long-term urologic outcomes begin to explore the issues of incontinence and quality among children and adults with neural tube defects of life. For adults with SB, urinary or fecal inconti- noted the type of neural tube defect influenced the nence does not appear to play a major determinant role urologic outcome (107). Neurogenic bladder was seen in health-related quality of life (120). in practically all those with myelomeningocele (MMC), with caudal regression syndrome (CRS) at >50% and Many patients with SCD receive regular urologic spinal lipoma (SL) at <50%. Vesicoureteral reflux was follow-up as children, but not necessarily as adults. most common in MMC, with CRS surprisingly close Adults with SCD who do not have urinary calculi or behind. The incidence of renal agenesis was highest urinary incontinence are often assumed to be urologi- in CRS. Subjects with SL were best controlled with CIC cally stable. However, many adults with SB (and likely and medications. SCI) have been found to have urologic abnormal- ities, such as abnormal renal ultrasound or elevated There have been a variety of surgical procedures serum Cr, that put them at increased risk of further to assist with acute and long-term management of renal problems, especially in the upper urinary tract neurogenic bladders developed and\/or promoted over (103). Many of these patients were also found to have the past 20 years in those with SCD. However, aside increased pressure (>40 cm H2O) in their bladder with from a few retrospective cohort studies regarding spe- the storage of urine at normal volumes. cific interventions (108\u2013110), there are no large or ran- domized controlled studies to identify best treatment Musculoskeletal strategies or factors that may indicate the procedure of choice (111,112). Furthermore, there are no reports of Level of motor function and musculoskeletal abnor- the effectiveness of surgical interventions over a life- malities are typically the areas of concern during time. A small study from a center in British Columbia growth and development, and often changes are not noted no significant increase in health-related quality anticipated during adult years. Pain is a common com- of life in SB patients who underwent reconstruction for plaint, and may be related to musculoskeletal issues, incontinence, compared to those who did not (113). although in SB, tethered cord must be considered. Moderate hypertension and proteinuria can also Overuse syndromes are common for wheelchair increase the risk of progression of renal dysfunction, users, and have been identified in adults with SCI at eventually leading to chronic renal failure in patients shoulders, wrists, and hands. In a comparison with with SB. For this reason, as well as the cardiovascu- adult wheelchair users, those with childhood-onset lar protective effects, even moderately elevated blood disabilities had fewer shoulder pain complaints than pressure should be treated. An angiotensin-converting those with adult-onset disabilities, even though life- enzyme (ACE) inhibitor should be considered, except styles were no different (121). Shoulder pain in adults in cases of advanced renal disease, due to the risk of and adolescents with SB is not as common as in adult- hyperkalemia and further advancement of the renal onset SCI wheelchair users, although older SB subjects disease (114). There are increasing numbers of chil- had more pain than younger ones (122). It is important dren with SB who have undergone renal transplanta- to identify the risk for shoulder pain, recognize the tion, with or without lower urinary tract reconstruction onset, evaluate, and treat appropriately.","442 Pediatric Rehabilitation Scoliosis is common in SB, and is a common con- patients with a higher level of defect have more of a risk tracture noted in adults. It rarely progresses in adult- for fractures (125,126). Most of the fractures reported hood. Spinal fusion has usually been performed prior in SB involved the tibia or femur, with 75% occurring to adulthood, but does not appear to improve the qual- in children after casting for an orthopedic procedure ity of life for those with SB (69). A combined anterior (127). Postorthopedic procedure and fracture manage- and posterior approach is reported to be more effec- ment must be tailored to the situation. Environmental tive in older adolescents and adults with pelvic obliq- modifications to prevent fractures may be more effec- uity (123). Seating difficulties, back pain, and pressure tive than pharmacologic interventions (126). ulcers arise from the scoliosis and pelvic deformities. Adults with SB report back pain less frequently than Neurologic those with SCI, although, in general, pain complaints increased with age. In adults with SB, back pain may Adults with SB are at an increased risk for neuro- presage tethered cord. logic complications because of the pathophysiology of their disability. Among the neurologic abnormal- Hip dislocation is related to thoracic or high lum- ities seen in SB, the most common for which to mon- bar neurologic-level abnormalities, and hip contrac- itor are hydrocephalus, Arnold-Chiari malformation\/ tures notable in high neurologic level, but also in hydrosyringomelia complex, and tethered spinal cord. thoracic and high lumbar levels (124). There are no The vast majority with hydrocephalus have some form published reports of hip or knee pain in adults with of shunting, possibly contributing to the increased SB, although this should not be unexpected in those survival rates seen today. However, shunt malfunc- who walk, given muscle imbalances and poor skeletal tions are not uncommon, are often unsuspected, and alignment. Charcot joints can be seen given the lack of can lead to significant morbidity and mortality (128). sensation and muscle imbalances, especially in adults Recommendation is that adults with SB have routine with SB, especially with lower-level defects (95). neurologic evaluations and periodic computed tomog- raphy (CT) scans to monitor the shunt (44), with report Osteoporosis with associated fractures has become that only 40% of adults with SB with a shunt have an area of interest and evaluation. For adults with SB, regular follow-up (129). Symptoms often seen with a the high incidence of renal dysfunction is an added shunt malfunction include headache, vomiting, leth- component for osteoporosis, given that renal dysfunc- argy, or change in mental status, with other neurologic tion can lead to impaired bone mineralization (105). sequelae also possible (44). Chronic headaches may be Renal dysfunction can also lead to metabolic acidosis seen in adults with SB, and recurrent hydrocephalus as well as hyperparathyroidism; there may also be or shunt malfunction must be excluded through intra- hyperphosphatemia, which can enhance the second- cranial pressure (ICP) monitoring if necessary (130). In ary hyperparathyroidism. This often necessitates the the absence of increased pressure, further treatment requirement for a low-phosphate diet and may also options should be considered for pain management. include taking phosphate-binding agents. For those Presence of hydrocephalus is associated with more with SB, bone mineral density is one to two standard dependence for self-care (including bladder and bowel deviations below the normal population, without a care), for mobility, and for communication and cogni- difference between ambulatory and nonambulatory tive assist into adulthood (96). patients (125). Treatment is not definitive. There are proponents for managing with calcium and vitamin D Adults with SB can have worsening neurologic and\/or using bisphosphonates, although no long-term symptoms from progression of an Arnold-Chiari mal- information is available. Continued walking with mus- formation, with or without the hydrosyringomyelia cle activity and weight bearing has a positive effect on complex. Presenting symptoms in adults with SB may bone mineral density in those with SB (125). Again, not be those seen typically with brainstem compres- long-term follow-up is not available to identify dosing sion, but may include upper limb weakness, sensory to achieve and maintain improvements. symptoms or reflex changes, ataxia, and lower cranial nerve palsies (94). Outcomes postsurgical intervention Fractures may be more concerning than the risk vary, including some level of recovery, stabilization of factor of osteoporosis. Few studies detail incidence symptoms, further deterioration, and even death. and prevalence. In an SB program cohort in upstate New York, where the vast majority is adults and Tethering of the spinal cord can be seen at any late adolescents, the overall fracture prevalence was age for those with SB who report changes in bladder 200\/1,000, most common during adolescence and or bowel habits, increase in leg weakness, change in least likely during adulthood. In comparing adult and sensory level, onset or increase of spasticity, report of childhood fractures, there was no significance to sex, pain (usually backache), or progression of deformities. body mass index (BMI), defect level, functional inde- In adults, an antecedent event such as trauma to the pendence, shunted hydrocephalus, epilepsy, or other back or buttocks often initiates symptoms. Prominent congenital anomalies (126). It has also been noted that","Chapter 15 Aging With Pediatric Onset Disability and Diseases 443 changes for adults are diffuse leg pain with referral to diarrhea may continue through adulthood; megaco- the anorectal area, and changes in bladder or bowel lon can develop if management is inadequate. It has habits, often difficult to detect given reconstructive been noted that assistance is commonly required for surgeries; progressive deformity usually is not noted, bowel management in adulthood (96,118,138). Bowel as is reported in children (131). Studies report that continence is often difficult to achieve, and lack of tethering, cord thinning, lipomas, cavities within the continence can influence ability to participate in com- cord, and diastematomyelia are common in this popu- munity activities. Of concern is assuring appropri- lation, with or without symptoms, so identification on ate evaluation and management of acute abdominal scan may not be definitive (132). Treatment consists symptoms; a case series of children and young adults of conservative management of symptoms with mon- notes etiologies included underlying neurogenic blad- itoring or neurosurgical intervention. Neurosurgical der or bowel, shunt, and complications from previous intervention is usually associated with improvement surgeries and a substantial mortality rate (139). in pain, urinary symptoms, and weakness, and poorer outcomes are associated with repeat procedures (133); Latex sensitization\/allergy is an important issue however, not all outcomes are good. for adults with SB, and the rate may be higher for adults than children (140). The risk of sensitization increases Epilepsy may remain an active problem in adult- with more surgical procedures being performed (141); hood for those with SB. Seizures are associated with the percentage of patients sensitized to latex ranges shunts. Most series identify program cohorts with from 2.97% to 64.5%. Radioallergosorbent testing has <15% requiring active seizure management with anti- been found to be more sensitive with a higher negative convulsants (92,94). predictive value and more accurate than skin prick testing. The prevalence of latex allergy in the SB popu- Additional Medical Conditions lation is almost 19%, while the prevalence of latex sen- sitization is 32.4% (141); therefore, every effort should Pulmonary conditions may be seen in adults with be made to limit exposure to latex. childhood-onset SCD, although is not typically reported in SB cohorts. Restrictive lung disease occurs Lymphedema is not reported in any large cohorts; as a consequence of scoliosis, and decreasing pulmo- however, it is clinically present and often associated nary function with age in the general population is with pressure ulcers. Simple over-the-counter com- well documented. For adults with SB, changing pul- pression garments are not useful, and most adults monary function may indicate further neurologic pro- with SB are unable to apply daily Ace wraps. The gression of a Chiari malformation. edema is often responsive to lymphedema wrapping followed by tailored compression garments. There are Obesity is a reported medical condition in motor two reports of severe and unresponsive lymphedema disabilities in general. It is commonly seen clinically in adult women with SB\u2014one responsive to suction- in an SB population, although it is not mentioned in assisted lipectomy (142), and the other progressed to a reported series of adults with SB. There are higher lev- diagnosis of lipedema, which has no successful treat- els of body fat in adults with SB who do not walk (134), ment regimen (143). and there is an association of increased body fat with previous hydrocephalus (135). Obesity can often be an Sexual Functioning associated factor with onset and management of pres- sure ulcers. Appropriate nutrition and adequate exer- The number of adults with SB and childhood-onset SCI cise and activity should be a lifelong goal in persons are increasing; therefore, the health care community with disabilities. can no longer ignore dealing with the medical and social issues of sexuality (144). A recent report noted that sex- Pressure ulcers are a commonly occurring sec- ual education was received at school, and far less at ondary condition in adults with SCD related to their home or by health professionals (145). Urinary incon- impaired protective sensation. For adults with SB, there tinence may limit sexual participation (145), although is an association with higher level and may be an asso- this is not a consistent report (146). Higher neurologic ciation with hydrocephalus (136,137). Osteomyelitis is level and presence of hydrocephalus was associated with a complication of recurrent or chronic pressure sores, less participation for both genders, but more problems and may ultimately require amputation for manage- with sexual functioning for men (137,145). There is no ment (118). published data regarding contraception, but for women, contraception or suppression can be offered considering Gastrointestinal conditions can be seen in adults risks (eg, thrombotic risk, lack of sensation for intrauter- with childhood-onset SCD. Usually, they are chronic ine devices [IUDs]), side effects, and need for follow-up rather than new or late-onset problems, unless related (147). Sexual education should be offered, with consider- to progressive neurologic conditions. Adults with SB ation for cognitive impairments when appropriate. also report problems with fecal incontinence in about 50% of reported cohorts (92,137). Constipation and","444 Pediatric Rehabilitation Many men with SB are able to achieve erections, with comparable functional levels incurred through but only about 53% are able to ejaculate (144). As antic- SCI as adults (150). More specifically, for those injured ipated, a lower defect gives men a greater chance of at a young age with incomplete injuries and minimal being able to sustain an erection, and there are normal deficits, there is about an 83% chance of normal life testosterone levels. Erectile dysfunction is treatable expectancy, and for those with high cervical injuries with medications, although men with SB in a study without ventilator dependence, the estimate is about did have some adverse effects after taking sildenafil, 50% of normal. including dyspepsia, nausea, headache, flushing and nasal congestion, hematologic changes, and UTI. The Life Satisfaction dyspepsia was treated with antacids, and the UTI was treated with antibiotics. The remainder of the adverse Adults with childhood-onset SCI show relatively high events did not require treatment (148). satisfaction with life and relate this to independent liv- ing, education, income, satisfaction with employment, Women with SB had fewer problems with sexual and social\/recreation opportunities (151,152). Medical functioning and were able to maintain pregnancies. complications adversely affect satisfaction, especially Arata reported that there was no increase in back pain, presence of pressure ulcers, severe UTIs, and spastic- no changes in neurologic or motor function, and no ity (152,153). Those with paraplegia are more satisfied changes in bowel or bladder function during or fol- than those with tetraplegia, and there appears to be lowing pregnancy (149). There were two commonly no gender difference (151). Depression symptoms have seen secondary conditions during pregnancy: UTI\u2014 been reported in adults with childhood-onset SCI, and but only in women who did not have normal voiding are associated with medical complications, social par- patterns\u2014and pressure ulcers sometimes requiring ticipation, and incomplete injury (154). Life satisfac- hospitalization. Women with SB also had more emer- tion is not associated with level of injury, age at injury, gent and elective C-sections than in the normal popu- or years with disability (152). lation. Women with SB were also found to have more antenatal admissions than women without SB, and Of interest is that adults with childhood-onset SCI it was noted that women with SB using wheelchairs self-perceptions are not reported to be as significantly exclusively had an average of 2.8 admissions antena- altered as clinicians anticipate (155,156) and, therefore, tally per pregnancy, with an average stay of 25.8 days, are enriched by services and providers that emphasize while women with SB who walked had an average of education, employment, and long-term health man- 1.9 admissions antenatally per pregnancy, with an agement (152). average stay of 17.3 days. More women with SB are admitted with preeclampsia than in the normal popu- Urology\/Nephrology lation, but given the incidence of renal dysfunction in this population, the prevalence is not overly high (149). The most common reported health complication for Further study is needed to fully address the possible adults with childhood-onset SCI was UTI (157). Typical complications of pregnancy and childbirth in patients strategies for management of neurogenic bladders are with SB. Pregnant women with SB may be evaluated used, as previously noted, and CIC continues to be the through a high-risk pregnancy service. typical management. Adults with childhood-onset SCI also frequently receive reconstructive lower tract sur- There is no information specifically regarding typ- geries; however, the decision factors determining best ical gynecologic screening and prevention practices for treatment options have not been determined. There women with childhood-onset SCD; however, national are studies reviewing specific interventions (108), but data regarding women with mobility impairments, there is no information regarding long-term effective- especially those requiring use of a wheelchair, clearly ness of surgical options. demonstrate minimal participation, likely due to envi- ronmental and attitudinal barriers. Pregnant women Adults with childhood-onset SCI have some asso- with childhood-onset SCD should be at least evaluated ciation of urologic complications that relate to age or through a high-risk pregnancy service. years with disability, and consequently, regular urologic follow-up is recommended. In a large study of adults Childhood-Onset Spinal Cord Injury followed at Shriners Hospital for Children in Chicago, Vogel reports older age at interview and longer years Mortality with disability were associated with orchitis or epidid- ymitis (157). Also, greater impairment was related to Using data from the National Spinal Cord Injury UTI, severe UTI, and renal stones. Severe UTIs were Statistical Center over a 30-year period, it has been also related to poor life satisfaction (153). Although not determined that life expectancy for adults injured as reported in this cohort, bladder cancer and pseudotu- children appears to be slightly lower than that of those mors of the bladder may also be present.","Chapter 15 Aging With Pediatric Onset Disability and Diseases 445 Musculoskeletal AD is associated with greater neurologic impairment and is a common health condition for adults with For adults with childhood-onset SCI, pain at any childhood-onset SCI (157). Spasticity is seen in >50%, site was the most common complaint, and shoulder older age at injury is associated with spasticity, and pain was noted in almost half of the respondents in longer years postinjury notes spasticity or neurologic interviews, as reported by Vogel at al (158). As was changes (158). Monitoring for changes in function and noted earlier, overuse syndromes must be considered, adjustment to spasticity or other management must be especially at the shoulder. In general, for adults with part of routine medical care, with consideration for all childhood-onset SCI, longer years with disability and possible options, including injections, pain manage- increasing age are associated with shoulder pain (158). ment, medications, and surgical considerations. Etiology must be identified, and evaluation and treat- ment are essential. An outpatient physical therapy pro- Additional Medical Conditions gram or a home exercise program for shoulder pain, with or without impingement, in SCI (159) have been Pulmonary conditions may be seen in adults with shown to be effective in pain management. childhood-onset SCI. Restrictive lung disease occurs as a consequence of scoliosis, and the addition of weak- For adults with childhood-onset SCI, younger age ness or paralysis of secondary respiratory muscles may at injury and longer years with disability has a correla- further increase risk for recurrent respiratory infec- tion with scoliosis (158,160). More severe and frequent tions (150). Survival for childhood-onset SCI requiring scoliosis has been reported in paraplegia and complete ventilator support has improved in recent years, with lesions, and lordosis has been noted to be greater in reported survival up to 23 years (163). Deaths in this paraplegia and incomplete lesions (160). There is no cohort were related to respiratory complications, fol- evidence that bony injury at the time of childhood- lowed by unknown and suicide. There have been rare onset SCI influences the development of scoliosis or unscheduled hospitalizations, and life satisfaction is lordosis (161). associated with better mental health. For adults with childhood-onset SCI, younger age Obesity is a reported medical condition in motor at injury and longer years with disability were asso- disabilities in general, but it is not mentioned in several ciated with hip subluxation, and older age at injury series of adults with childhood-onset SCI. Appropriate was associated with elbow and ankle pain (158). Back nutrition and adequate exercise and activity should be pain may be seen in about 20% of patients unrelated a lifelong goal in persons with disabilities. to scoliosis, and ankle pain and elbow contractures are associated with tetraplegia, and hip contractures Pressure ulcers were reported in just less than with paraplegia (158). For those who walk, presence 50% of adults with childhood-onset SCI, were more of hip or knee pain should be questioned, and for any common in men, and more common in greater neuro- pain complaint, appropriate workup and management logic impairment (157). should ensue. Gastrointestinal conditions are not common, other There are no reports detailing osteoporosis in than neurogenic bowel\u2013related issues. Bowel inconti- adults with childhood-onset SCI; however, there is sci- nence is reported in >50% of adults with childhood- entific research that identifies osteoporosis as a com- onset SCI, and is seen with older age and greater mon secondary condition in SCI. As noted, the most impairment, although not with increasing years with effective treatment has not been established, and dos- disability. ing parameters for medications or other strategies are unknown. Case series have advocated for the use of Latex sensitization\/allergy is seen in SCI, but cycling with functional electrical stimulation (FES) to seemingly not as frequently as SB. It is unclear what improve bone mineral density (162). the incidence of latex allergy is in the childhood-onset SCI population, although it is known that women more Adults with childhood-onset SCI report fractures commonly report a latex allergy (157). associated with increasing age and longer years with disability (158). Those with lower cervical injuries Osteoporosis. There is no published data about tend to have more pathological fractures than the osteoporosis in adults with childhood-onset SCI dif- other groups. fering from adults with SCI. Treatments studied have included bisphosphonates and functional electrical Neurologic stimulation (FES) exercise, although there is no defin- itive treatment suggested by the research findings. Neurologic sequelae for adults with childhood-onset Fractures are the complication, and are reported with SCI appear to be limited by report in the literature. The increasing age (158). Another bony deformity, hetero- presence of autonomic dysreflexia (AD) is not related to topic ossification (HO), is not reported as significant in increasing age, age at injury, or years with a disability. this population, and decreased with age in a study of adults with SCI (164).","446 Pediatric Rehabilitation Sexual Functioning deficits, muscle weakness, or heart disease. Typical surveillance for these disorders is important to main- There are less data about men and women with tain ambulation status. childhood-onset SCI. Although the general informa- tion available about adults with SCI can be helpful, it Intellectual Disabilities is not clear if it can be generalized. It is known that semen quality decreases at about two weeks postin- Intellectual disability is a common reason for disabil- jury, which could imply decreased fertility for adult ity in childhood, although less prominent in adult men with childhood-onset SCI (165). Fertility is also surveillance. People with intellectual disabilities expe- affected by bladder care (166). rience age-related health impairments at a higher rate and earlier age than people without disability (172). There are no menstrual cycle difficulties known Depending on the etiology of their disability, they for women with childhood-onset SCI (167). A multi- may be at much higher risk for both secondary condi- center study of women\u2019s self-reported reproductive tions and comorbidities. These conditions can be life- health after SCI, likely adult-onset injuries, reported threatening or life-altering. Some may be prevented or complications from pregnancy, labor, and delivery to treated if identified early. Down syndrome (DS) will be more frequent than what was noted preinjury, and be discussed as a separate entity, as more is known delivered babies of low birth weight (168). Women about aging with this condition. Strategies for mini- reported increased bladder spasms, muscle spasms, mizing functional limitations will be highlighted. and autonomic symptoms at some time during their Rehabilitation surveillance and treatments will be dis- menstrual cycle. Experience of orgasms and methods cussed (see Table 15.2). of contraception varied. The effects of menopause are unknown. Intellectual Disability There is no specific information about typical Individuals with intellectual disability (ID) are living gynecologic screening and prevention practices for longer and experiencing most of the same illnesses women with childhood-onset SCI; however, national as the general population (173). Their life expectancy data concerning women with mobility impairments, remains somewhat less than the general population, especially those requiring use of a wheelchair, clearly but has steadily increased with the move away from demonstrate minimal participation likely due to envi- institutionalized care (174). Community-based health ronmental and attitudinal barriers. Risks for use of care for people with ID is not well organized, and peo- contraception options are not known; however, com- ple with ID experience poorer health than the general bined hormone oral therapy carries a risk for throm- population (175). bophlebitis; progestin-only medications have early irregular bleeding and long-term suppression effects; Cardiovascular and intrauterine devices with lack of sensation require vigilance for correct placement and risk of rare compli- Janicki and colleagues noted that cardiovascular dis- cations such as perforation, infection, or ectopic preg- ease (CVD) and respiratory diseases were more com- nancy (147). Given the information self-reported by mon causes of death in the elderly with ID than in women with SCI, pregnant women should be at least the general population, with cancers in a less promi- evaluated through a high-risk pregnancy service. nent role (173). Although there have been discussions of significant rates of chronic health conditions and Limb Deficiency general poor health for adults with developmental dis- abilities, more recent studies of adults receiving state Pediatric-onset limb deficiency is not uncommon, or national support in New York state, Taiwan, and with 4\/10,000 in upper extremity congenital limb defi- Israel (82,83,176) note gradual increases in health con- ciency alone. In addition, lower extremity hemimelia, ditions, but not with higher incidence than in the gen- traumatic amputations, and childhood cancers are eral population, and in some cases lower. associated with pediatric limb deficiency. Very little is known about aging with this disability. However, Obesity certain comorbidities and secondary conditions are typical for this group (see Table 15.2). Weight control In a cross-disability study of a South Carolina primary is important to prevent osteoarthritis (169,170). One care practice that included almost 50% adults with author describes increased velocity and lower effort developmental disabilities (DD), there was a lower in elderly amputees if a locked knee is used (171). odds ratio for coronary artery disease, cancer, and obe- Changes in gait or use of upper limb prostheses with sity for adults with DD in comparison to those without aging in this population may be due to a variety of typical disorders of aging, including arthritis, sensory","Chapter 15 Aging With Pediatric Onset Disability and Diseases 447 disabilities and compared to other disability groups more prevalent in the elderly population with ID. Each (177). Although obesity was reported as low in the of these groups also had high numbers of health comor- South Carolina study, other studies report obesity as bidities, such as CVD, sensory impairment, and mobil- being more common in adults with developmental dis- ity problems. Researchers note that life events, such as abilities. Obesity in people with ID is higher, compared relocation, were more frequent in adults with ID than in to those age-matched without ID (35.4% vs 20.6% in comparison groups (191). Medication review is a prior- one survey) (178). Other researchers have found twice ity for clinicians treating people with ID. Polypharmacy as many people with ID to be obese as those without is a significant problem for people who may not have ID within the same community (179,180). Those with adequate understanding of the need to report side mild ID have more obesity than those with severe ID, effects or efficacy of medications. Medications should and there can be a move out of the obesity state (181). not be prescribed unless a system is in place to ensure The combination of increased obesity and mortality compliance, safety, and monitoring of efficacy (183). due to CVD lead to a recommendation of increased sur- Surveillance for mental health problems in aging peo- veillance and prevention strategies for obesity-related ple with ID should be a priority, along with treatment disease. of physical comorbidities, which may contribute to or appear as mental health concerns. Respiratory Sexual Functioning Several authors describe respiratory ailments as impor- tant factors in morbidity and mortality of aging adults People with ID are often not afforded typical educa- with ID (172,173,182). Janicki and colleagues identified tion, contraception options, or sexual health screen- pneumonia as the most prevalent cause of death due to ing. They face a high risk of sexual abuse, are unaware respiratory illness and second only to CVD (173). Sleep of protection from sexually transmitted diseases, and apnea due to obesity is mentioned as a comorbidity are generally unsupported in attaining healthy sex- and may require separate screening or sleep studies. ual relationships (147,193,194). Women are often pre- scribed suppression therapy (194,195). Sterilization for Health Maintenance women with ID is more common abroad, and related to severity and living arrangement (196). Women and People with ID require the same screening for cancers, men with ID can be provided with education and sup- diabetes, hyperlipidemia, hypertension, bone density, port for sexual functioning, and regular health screen- and ophthalmologic and hearing disorders as the gen- ings can be accomplished with modifications and eral population. Communication about the results of support (147). these screenings and plans for treatment of any abnor- malities may need to be through a proxy. Prevention Down Syndrome strategies for diseases related to obesity may need to start earlier than in the general population. Preexisting More than half of people with Down syndrome (DS) conditions of epilepsy and poor oral health should be will survive to age 50, and half of those will be alive monitored closely (183). GERD and Helicobacter pylori at age 65 (197). Most people with DS are living in the infection is increased in prevalence and undertreated community with family or in supported living. They in people with ID (184,185). Symptoms of GERD should require increased health care surveillance as they age be queried in people with ID and treatment under- due to higher prevalence of numerous clinical condi- taken, as with the general population. Osteoporosis tions. Access to appropriate health care may prove also is more prevalent in people with ID, with precip- difficult for people with DS, as they may have diffi- itating factors of small size, hypogonadism, and anti- culty with communication or behavior and typical pri- convulsant therapy (186\u2013188). Fractures are associated mary care practices may not meet their needs. Specific with frequency of falling. Screening for osteoporosis health screening programs have shown a dramatic and falling should commence during early adulthood, increase in recognition of unmet health care needs with follow-up depending on the results. (23). Rehabilitation clinicians can assist families and primary care physicians to provide optimal mainte- Mental Health nance of function throughout life. Mental health impairments are prevalent in elderly Mental Health people with intellectual disability. Estimates vary from 20% to 70%, depending on which assessments were used Mental health problems in people with DS have been and the exact population studied (189\u2013192). Dementia, well described in the literature. An elderly (>65) depression and general psychiatric symptoms are all group was well described by Cooper and colleagues","448 Pediatric Rehabilitation as having increased dementia, anxiety, and depres- multifactorial, with obesity (225), central (brainstem sion when compared to a younger group (190,198). respiratory control) mechanisms (222), and obstruc- Symptoms of Alzheimer\u2019s may be seen as early as age tive (221,223,226) sources all implicated. Sleep apnea 35 and will be noted in 75% of people with DS by age is associated with worsened cognitive skills (227), 60 (191). A variety of causes have been postulated for and may be successfully treated in a variety of ways the high incidence of Alzheimer\u2019s\/dementia in peo- (226,228,229). A sleep study is indicated to identify the ple with DS, including antioxidant stress (199) lower cause and therefore predict the successful treatment bioavailable estradiol in women (200), and decreased for sleep apnea. alpha and beta secretase activity (201). Musculoskeletal Treatable comorbidities, which may look like Alzheimer\u2019s, must be ruled out. These include hypo- Premature arthritis has been reported in adolescents thyroidism, visual and hearing impairments, depres- and adults with Down syndrome and may be associ- sion, and epilepsy, all of which are significantly ated with joint subluxations and dislocations (197,230). more common in DS than in other populations with Hip instability may occur or worsen in adults with Alzheimer\u2019s (202). Likewise, systemic illness, infec- DS and is associated with decreased ambulation sta- tion, drug effects, and alcoholism must also be elim- tus (231). Foot pain and arthritis may be associated inated as possible treatable causes of Alzheimer\u2019s with severe pronation and atypical gait; however, symptoms (203). very little research has been done in this area (232). X-rays are indicated if ambulation status deteriorates. Depression may cause decreased function in people Treatment may begin with NSAIDs, but further evalu- with Down syndrome (177,198,203,204). Experiences ation and possible referral is indicated if typical arthri- of loss may trigger depression, as may changes in work tis pain relief strategies are not sufficient to maintain or living situations. Depression may be treated with function. counseling; however, training or experience with this population will be needed for counseling to be effec- Osteoporosis is also more common in adults with tive. Treatment may also include medications. The DS and is found in both men and women at a signif- use of selective serotonin reuptake inhibitors (SSRIs) icantly younger age than in the general population in DS has been anecdotally described, but no ran- (233). Long bone and vertebral compression frac- domized controlled trials have been reported to date tures are common (234). Decreased physical activity, (203,205\u2013208). short stature, early menopause, low muscle tone, and increased incidence of thyroid disease may all be fac- Endocrine System tors in osteoporosis in DS (200). Thyroid disease is well described as a comorbid- Atlantoaxial Instability ity of DS (23,172,173,189\u2013191,197,202\u2013204,209\u2013212). Hypothyroidism is found in 15% to 50% of adults with One to two percent of individuals with DS will have DS (197,202,203,213\u2013215). Thyroid-stimulating hor- cervical subluxation or symptomatic atlantoaxial mone levels should be assessed annually in patients instability (AI) (235). Routine monitoring via x-ray is with DS (203). no longer recommended, but vigilance for progression is recommended. Concerning symptoms include new Diabetes mellitus may have a higher prevalence in torticollis, weakness, neck pain, change in gait, change adults with DS, but is rarely discussed in the literature in bowel or bladder function, increased reflexes, or (202,203,216,217). McDermott and colleagues found other symptoms of spinal cord compression (236). fewer developmentally disabled adults with diabetes Presentation of these symptoms requires immediate than control adults in a large primary care practice stabilization and referral for surgery consideration (177). Typical yearly testing and treatment as needed (197,235,236). Outcomes from surgery are not always should suffice for surveillance. acceptable (237,238). Otolaryngology Cardiac Hearing loss is extremely common in people with Nearly half of infants born with Down syndrome will Down\u2019s syndrome and may not develop until have a structural heart anomaly. Most of the typi- adulthoo(189,197,203,218,219). Poor hearing may exac- cal congenital heart abnormalities will have been erbate preexisting communication difficulties and corrected in infancy. Increased incidence of mitral present as behavior problems. Auditory testing is rec- valve prolapse in adults with DS has been reported ommended at least every two years in adults with DS. (197,203,209). Careful auscultation should reveal any Sleep apnea is also a common problem for adults with Down syndrome (220\u2013224). The cause is likely","Chapter 15 Aging With Pediatric Onset Disability and Diseases 449 change in heart murmurs, and electrocardiogram and symptoms and compliance with health recommenda- chest x-ray can follow. tions (172,203,225,248,249,261). Sexual health should not be ignored, and often contraception or suppression Cardiovascular disease (CVD) is not well studied is prescribed for women for hygiene problems with in people with Down syndrome. As people with DS menstrual cycles (147,195). live longer, become more obese, and less active, it is reasonable to expect to see increasing rates of CVD Williams\u2019 Syndrome (173,225,239). Several authors have noted decreased cardiovascular capacity in people with DS (240\u2013244). Williams\u2019 syndrome (WS) is caused by a gene deletion A 2005 Cochrane review of exercise training programs on chromosome 7. It is rare, occurring in 1 of 20,000 for people with DS revealed only two small trials of live births (262). Devenny and colleagues have been good quality. Of these, only maximal treadmill grade following a group of 15 adults with WS, some of whom was improved after the training program. Other stud- have participated in a 15-year longitudinal study on ies have investigated components of fitness such as aging in adults with ID. The participants with WS leg strength and capacity, as noted previously. Small demonstrated early and rapid decline in long-term epi- uncontrolled trials not included in the Cochrane sodic memory not found in other adults with ID. Verbal review have shown only limited aerobic improvement short-term memory was better than their peers with ID with exercise training programs (243,245). and did not decline with age (262,263). No associa- tion was found with physical or mental comorbidities. Obesity Because Williams syndrome has only been clearly described within the current generation of adults, few Obesity is a lifelong issue for many people with Down people have been extensively studied, and we do not syndrome. As many as 70% of adults with DS are yet know the causes of the apparent precocious aging reported to be obese (225,246,247). Health promotion noted in this population. and group exercise classes have been successful at sig- nificantly reducing body fat percentages in short-term TRANSITIONS AND ACCESS programs (240,241,243,245,248\u2013250). TO HEALTH CARE Sexual Functioning Transition of Care to Adult Services There is little published information regarding sexual Improved medical care and increasing numbers of functioning in adults with DS. It has long been held adults with childhood-onset disabilities has lead to that males are infertile and females are fertile or sub- much interest and concern about the transitioning of fertile based on histology of gonads and serum levels care of young adults from a family-centered pediat- (251,252). There are case reports of men and reports of ric approach to a self-directed adult care model (2). small series of women who have been fertile (253,254). Pediatricians often will maintain care for their The male offspring are reported to have no abnormal- patients well into adulthood, especially for those with ities, congenital or genetic. In contrast, the female complex medical conditions (264). A consensus pol- offspring are reported to have DS, be chromosomally icy statement, adopted by the American Academy of normal, or have other congenital defects or ID. The Pediatrics, American Academy of Family Physicians, need for education and counseling, monitoring for and the American College of Physicians\u2014American sexual abuse, and social support is obvious. Society of Internal Medicine, states that the transi- tion of care should \u201cmaximize lifelong functioning Health Maintenance and potential through the provision of high-quality, developmentally appropriate health care services that People with Down syndrome require the usual screen- continues uninterrupted as the individual moves from ings for testicular and cervical or breast cancer and adolescence to adulthood.\u201d (265) Barriers to transi- hypertension. Celiac disease is now recognized as a tions cited include lack of adult provider training, common condition associated with DS, and monitor- poor communication between pediatric and adult ing should be a part of health maintenance (255,256). providers, and need for self-direction navigating the Dental health is important, as gingivitis and peri- adult system (266). There have also been suggestions odontal disease are more common in people with DS for specific elements to support a transition, such as (257\u2013260). Cataracts and keratoconus both occur with preparation, flexible timing, care coordination, transi- increased frequency in people with DS. Regular oph- tion clinic visits, and interested adult care providers; thalmologic examinations are indicated to evaluate for however, this remains theoretic (267). At present, the these conditions. Health care screening and promotion programs have demonstrated improved detection of","450 Pediatric Rehabilitation science is at an early stage of development (266,268). examination and procedure tables continue to be avail- Adolescents with early-onset and chronic health care able on only a limited basis. Attitudinal barriers are needs have received an organized level of care, and more difficult to remedy, and involve both consumers maintaining coordination of often complex care is an and providers. Rehabilitation clinicians may need to ask important part of quality health care over a lifetime. more direct questions of their patients regarding second- ary conditions and additional health concerns to better There have been reports of successful transition identify conditions and begin management. Physiatrists of service models. Successes related to planned and can act as a resource for primary care providers, who evaluated transitions (269), personal health records likely have limited knowledge regarding persons with management (269,270), and provision of education on lifelong disabilities. Consumers with communication or health and needs (271). There remain questions regard- cognitive impairments (eg, hearing impairment, speech ing shared responsibilities for the transition (272\u2013274), production impairment, brain injury, ID) may need need for protocols (275), and timing for planning and more time to communicate, require an interpreter, or implementation. Pediatric physiatrists can often pro- require personal preparation time for the appointment vide the stability for this transition. Table 15.3 iden- in order to have their needs conveyed; modification of tifies challenges for transitioning health care from appointment times, with preplanning and written lists pediatric to adult systems of care (276). of concerns, can often be helpful. Consumers may seek help only late in the course of an acute medical condi- Access to Health Care tion or change because of previous difficulties manag- ing the system. Specifically, consumers report that their Access to health care for young adults has been prob- routine health care providers know little about their lematic for funding reasons as well as transition-of-care disability and its impact on health and function (43). difficulties. Lack of insurance has been highlighted, and is as common among young adults without disabilities Health and Wellness Agenda as those with disabilities, as noted through the National Health Information Survey (277). However, adults with As a result of the steady improvement in medical care disabilities had eight times greater odds of reporting and social support systems during the last 50 years, unmet health care needs and six times greater odds of persons with disabilities are healthy, conducting active having no usual source of care, compared to those with- and productive lives, and generally living longer. The out disabilities. The majority of young adults with dis- medical paradigm must now shift from that of illness abilities reported a gap in their insurance coverage, and and disease to one of health and wellness. The health many were uninsured over a three-year period (278). care delivery system must view persons with disabilities through a typical health maintenance and preventive Access also involves environment, attitudes, and medicine approach. This requires a change in attitudes systems. Architectural barriers have been addressed and care models. Both prevention and promotion strat- through the Americans with Disabilities Act, although egies should be employed: prevention of activities that accessible health care providers\u2019 offices and accessible lead to illness and disease (eg, smoking cessation, die- tary discretion, routine laboratory and examinations, 15.3 Characteristics That Affect protected sexual activity) and promotion of activities Successful Transition of Care* that improve general well-being (eg, stress management, exercise) adapted to meet individual requirements and Simple transition Complex transition performance (Table 15.4) (279,280). However, positive Single condition Multiple conditions health behaviors require social, health, and commu- Few medications Multiple medications or allergies nity resources. The more resources a person has, the No cognitive impairments Profound intellectual disability more likely that individual will engage in health pro- No physical impairments Physical impairments motion and protective behaviors (281). Again, access is No behavior concerns Serious behavioral issues an important issue. Availability of information in appro- Mentally healthy Mentally ill priate modalities and the education of consumers are Effective family support Family ineffective important. To participate in positive health behaviors, Few physician Multiple subspecialties involved one must be interested, be ready to make changes, have the needed resources, and have a supportive environ- consultants required In-home skilled nursing and ment. Early involvement of adolescents with mobility No nursing care needs special equipment and supplies impairments in health promotion activities may pave the way for maintaining these behaviors into adulthood. Adapted from Ref 282. Since musculoskeletal conditions are the most com- mon age-related changes and secondary conditions that","15.4 Health Preventive Screening Services* HEALTH CONDITION RECOMMENDATION FOR GENER AL POPUL ATION Hyper tension Immunizations >18 yrs and annually Cardiac, vascular diseases Follow schedule Men: >35 yrs; possibly 20 yrs with CAD risks Lipid Women: >45 yrs with CAD risks; possibly 20 yrs with risks Abdominal aortic aneurysm Men: age 65\u201375 yrs if ever smoked Cancer Colorectal Men and women, screening >50 yrs Women\u2019s health Breast Annual mammogram >40 yrs Clinical exam, every 3 yrs 20s\u201330s, annual >40 yrs Cervical Self-exam option >20 yrs MRI only with high risk, annually Prostate Screening begins 3 yrs postintercourse, not later 21 yrs Age 30 yrs, with 3 normal Pap tests, screen 2\u20133 yrs Metabolic >70 yrs, 3 normal Pap tests and no abnormals or risks may Obesity D\/C after total hysterectomy and no risks Diabetes mellitus Offer PSA and digital exams >50 yrs, not required High risk, test 40 yrs; if normal, begin routine 45 yrs Mental health >75 yrs not required Depression Screening for all, with counseling and behavior intervention Screening for asymptomatic sustained blood pressure >135 Screening if able to diagnose, treat, follow-up Dementia Insufficient data to recommend in general population Violence Not recommended for general population Tobacco use Recommend regular screening and offer cessation interven Exercise Unclear that screening is effective in the general population Aging Presbyopia, cataract, macular degeneration, and glaucoma Vision age\u2014unclear screening is effective Hearing >50 yrs, hearing decreases; unclear if screening is effective 451 CAD, coronary artery disease; MRI, magnetic resonance imaging; D\/C, discontinue; PSA, prostate-specific an Adapted from Ref 286.","N MODIFICATION NEEDED y discontinue None None None Accessible procedure environment Accessible procedure environment May need 1:1 assist Accessible office exam table and procedure environment May need 1:1 assist Accessible procedure environment May need 1:1 assist Office exam table accessibility ns offered Requires accessible scale 5\/80 mm Hg None tions May require modification to queries; requires support to n diagnose and treat increases with increasing Important to question in DS e High incidence of violence and abuse in disability; offer ntigen; DS, Down syndrome. opportunity to discuss None Exercise is an important activity for those with motor impairments; has been shown to be effective for improved performance, pain control, weight management Accessible examination Accessible examination","452 Pediatric Rehabilitation affect performance, it would seem most reasonable to physical performance and the musculoskeletal system. view typical physiatric strategies and interventions as Prevention strategies require knowledge of expected preventive management techniques. Use of adaptive changes, recognition of changes that alter function equipment, energy-conservation techniques, joint pro- and require intervention, and an understanding of tection, and ergonomic positioning may enhance func- interventions that positively impact on function. This tion, decrease musculoskeletal complaints, and possibly requires that a person with a disability have access to prevent or delay some functional changes. Personal atti- knowledgeable health care providers. Physiatrists may tudes (of the person with a mobility impairment or their offer that knowledge through direct clinical service or personal support system) may have to change before indirectly functioning as a resource in the community. a person with impaired mobility will consider such Environmental, communication, attitudinal, and sys- assistance or be supported in considering the value of tems barriers must be overcome in order for health employing supportive (less independent) techniques. care providers and people with disabilities to work together for the best possible outcomes. Exercise is a well-known health-promoting behav- ior, and its effects are positively demonstrated in per- It is time to reconsider the model of illness and dis- sons with disabilities (24,282\u2013286). Benefits of a regular ease for persons with lifelong disabilities. Particularly exercise program include improved fitness, weight in the realm of mobility, a health and wellness model reduction, improved mood, and improved sleep. It is should be developed. Use of prevention strategies must also known that persons must be judicious in partici- be considered in childhood and adolescence to address pating in exercise programs, given the issues of fatigue the more frequent secondary conditions. Programs of and pain. Of course, care must be taken in prescrib- fitness and exercise have been proven beneficial in non- ing exercise for persons with impaired mobility; they disabled groups and disability groups alike. Health pro- should participate in an appropriate program of exer- motion strategies should be employed for persons with cise or activity, especially keeping in mind their risk congenital and childhood-onset mobility impairments. factors for musculoskeletal injury. Jogging or running started by young adults without disabilities more often PEARLS resulted in discontinuation of exercise because of joint pain than for persons who started a similar exercise \u25a0 Most adults with early-onset disabilities are healthy program in their middle years, leading one to believe with aging. Significant or acute loss of function that long-term, high-impact exercise may result in should not be expected, and evaluation must ensue. pain. Aquatics programs can eliminate the wear and tear to joints. Adults with cerebral palsy tend to report \u25a0 Adults with early-onset disabilities view themselves perceived changes in balance and then fear of falling, as healthy, although this is dependent on the num- which usually improves with a general fitness program. ber of health conditions. Life satisfaction is usually Exercises, including strengthening exercises, are not not associated with disability. This is within the contraindicated for persons with spasticity. Generally, context of measurement instruments that have not adults and young adults with developmental disabili- been standardized for those with disabilities. ties do not participate in routine fitness or exercise pro- grams. This may be as much from limited knowledge in \u25a0 Urinary\/renal issues for adults with childhood-onset this area as from attitudes of care providers and persons SCD are of primary concern. However, management with disabilities relative to exercise as a self-directed, of pressure ulcers and lymphedema can be most nonmedical, or leisure activity. Consideration of exer- problematic. cise programs at home, in a health club, or as part of an individual recreation program (with or without modi- \u25a0 Consider newer tone management options to manage fications) must be initiated earlier than adulthood to pain or improve function, with concomitant therapy. achieve long-term participation. And, just as in the non- With decreased tone, additional focused therapy can disabled population, priorities for persons with mobility improve function. impairment should include exercise and fitness. \u25a0 Pain is common in adults with childhood-onset SUMMARY disabilities. All pain is not arthritis, and there can be many etiologies. Never miss the oppor- Adults with early-onset disabilities are generally tunity to question, evaluate, diagnose, and treat. healthy. Not all adults have serious health prob- Although most pain is musculoskeletal in origin, lems, and many now recognize the aging process as if there is no improvement, consider neurologi- a natural course of events. The most common age- cally based etiologies, such as stenosis, tethering, related changes and secondary conditions involve or entrapments. \u25a0 Exercise can improve performance, and any per- son with a disability can participate, with modifi- cations. 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Hum Genet. 231. Hresko MT, McCarthy JC, Goldberg MJ. Hip disease 1983;63:132\u2013138. in adults with Down syndrome. J Bone Joint Surg Br. 253. Sheridan R, Llerena J,Jr, Matkins S, Debenham P, 1993;75:604\u2013607. Cawood A, Bobrow M. Fertility in a male with trisomy 21. [see comment]. J Med Genet. 1989;26:294\u2013298. 232. Caselli MA, Cohen-Sobel E, Thompson J, Adler J, 254. Pradhan M, Dalal A, Khan F, Agrawal S. Fertility in men Gonzalez L. Biomechanical management of children and with Down syndrome: A case report. Fertility & Sterility. adolescents with Down syndrome. J Am Podiatr Med 2006;86:1765.e1\u20131765. Assoc. 1991;81:119\u2013127. 255. Zachor DA, Mroczek-Musulman E, Brown P. Prevalence of celiac disease in down syndrome in the united states. 233. Center J, Beange H, McElduff A. People with mental retar- J Pediatr Gastroenterol Nutr. 2000;31:275\u2013279. dation have an increased prevalence of osteoporosis: A 256. Bonamico M, Mariani P, Danesi HM, et al. Prevalence and population study. Am J Ment Retard. 1998;103:19\u201328. clinical picture of celiac disease in Italian Down syndrome patients: A multicenter study. J Pediatr Gastroenterol Nutr. 234. van Allen MI, Fung J, Jurenka SB. Health care concerns 2001;33:139\u2013143. and guidelines for adults with Down syndrome. Am J Med 257. Amaral Loureiro AC, Oliveira Costa F, Eustaquio da Genet. 1999;89:100\u2013110. Costa J. The impact of periodontal disease on the quality of life of individuals with Down syndrome. Downs Syndr 235. Msall ME, Reese ME, DiGaudio K, Griswold K, Granger Res Pract. 2007;12:50\u201354. CV, Cooke RE. Symptomatic atlantoaxial instability asso- ciated with medical and rehabilitative procedures in chil- dren with Down syndrome. Pediatrics. 1990;85:447\u2013449. 236. Nader-Sepahi A, Casey AT, Hayward R, Crockard HA, Thompson D. Symptomatic atlantoaxial instability in Down syndrome. J Neurosurg. 2005;103:231\u2013237. 237. Taggard DA, Menezes AH, Ryken TC. Treatment of down syndrome-associated craniovertebral junction abnormal- ities. J Neurosurg. 2000;93:205\u2013213. 238. Doyle JS, Lauerman WC, Wood KB, Krause DR. Complications and long-term outcome of upper cervical spine arthrodesis in patients with Down syndrome. Spine. 1996;21:1223\u20131231. 239. Esbensen AJ, Seltzer MM, Greenberg JS. Factors predict- ing mortality in midlife adults with and without Down","460 Pediatric Rehabilitation 258. Boyd D, Quick A, Murray C. The down syndrome patient 272. Geenen SJ, Powers LE, Sells W. Understanding the role of in dental practice. Part II: Clinical considerations. N Z health care providers during the transition of adolescents Dent J. 2004;100:4\u20139. with disabilities and special health care needs. J Adolesc Health. 2003;32:225\u2013233. 259. Morgan J. Why is periodontal disease more prevalent and more severe in people with Down syndrome?. Spec Care 273. Betz CL. Nurse\u2019s role in promoting health transitions for Dentist. 2007;27:196\u2013201. adolescents and young adults with developmental disabil- ities. Nurs Clin North Am. 2003;38:271\u2013289. 260. Nualart Grollmus ZC, Morales Chavez MC, Silvestre Donat FJ. Periodontal disease associated to systemic genetic 274. Rosenbaum P, Stewart D. Perspectives on transitions: disorders. Med Oral Pato Oral Cir Bucal. 2007;12:E211\u2013215. Rethinking services for children and youth with devel- opmental disabilities. Arch Phys Med Rehabil. 2007;88: 261. Melville CA, Finlayson J, Cooper SA, et al. Enhancing pri- 1080\u20131082. mary health care services for adults with intellectual dis- abilities. J Intellect Disabil Res. 2005;49:190\u2013198. 275. Por J, Golberg B, Lennox V, Burr P, Barrow J, Dennard L. Transition of care: Health care professionals\u2019 view. J Nurs 262. Devenny DA, Krinsky-McHale SJ, Kittler PM, Flory M, Manag. 2004;12:354\u2013361. Jenkins E, Brown WT. Age-associated memory changes in adults with Williams syndrome. Dev Neuropsychol. 276. Kelly AM, Kratz B, Bielski M, Rinehart PM. Implementing 2004;26:691\u2013706. transitions for youth with complex chronic conditions using the medical home model. Pediatrics. 2002;110:1322\u20131327. 263. Krinsky-McHale SJ, Kittler P, Brown WT, Jenkins EC, Devenny DA. Repetition priming in adults with Williams 277. Callahan ST, Cooper WO. Access to health care for young syndrome: Age-related dissociation between implicit and adults with disabling chronic conditions. Arch Pediatr explicit memory. Am J Ment Retard. 2005;110:482\u2013496. Adolesc Med. 2006;160:178\u2013182. 264. Burke R, Spoerri M, Price A, Cardosi AM, Flanagan P. 278. Callahan ST, Cooper WO. Continuity of health insurance Survey of primary care pediatricians on the transition and coverage among young adults with disabilities. Pediatrics. transfer of adolescents to adult health care. Clin Pediatr 2007;119:1175\u20131180. (Phila). 2008;47:347\u2013354. 279. Centers for Disease Control and Prevention. Economic 265. American Academy of Family Physicians and American costs associated with mental retardation, cerebral palsy, College of Physicians-American Society of Internal hearing loss, and vision impairment, United States, 2003. Medicine. A consensus statement on health care transi- MMWR Morb Mortal Wkly Rep. 2004;53:57\u201359. tions for young adults with special health care needs. Pediatr. 2002;110:1304\u20131306. 280. Agency for Healthcare Research and Quality. Health Prevention Services Task Force. Available at: http:\/\/www. 266. Betz CL. Transition of adolescents with special health care ahrq.gov\/CLINIC\/uspstfix.htm. needs: Review and analysis of the literature. Issues Compr Pediatr Nurs. 2004;27:179\u2013241. 281. Kulbok PP. Social resource, health resources, and preven- tive behaviors: Patterns and predictions. Public Health 267. Binks JA, Barden WS, Burke TA, Young NL. What do we Nursing. 1985;2:67\u201381. really know about the transition to adult-centered health care? A focus on cerebral palsy and spina bifida. Arch Phys 282. Rimmer JH, Rowland JL. Health promotion for people Med Rehabil. 2007;88:1064\u20131073. with disabilities: Implications for empowering the person and promoting disability-friendly environments. Journal 268. Stewart D, Stavness C, King G, Antle B, Law M. A crit- of Lifestyle Medicine. 2008:409. ical appraisal of literature reviews about the transition to adulthood for youth with disabilities. Phys Occup Ther 283. Kilmer DD. Response to resistive strengthening exercise Pediatr. 2006;26:5\u201324. training in humans with neuromuscular disease. Am J Phys Med Rehabil. 2002;81:S121\u2013126. 269. Sawyer SM, Collins N, Bryan D, Brown D, Hope MA, Bowes G. Young people with spina bifida: Transfer from 284. Petajan JH, White AT. Recommendations for physical paediatric to adult health care. Journal of Paediatrics & activity in patients with multiple sclerosis. Sports Medicine. Child Health. 1998;34:414\u2013417. 1999;27:179\u2013191. 270. Osterlund CS, Dosa NP, Arnott Smith C. Mother knows 285. Durstine JL, Painter P, Franklin BA, Morgan D, Pitetti KH, best: Medical record management for patients with Roberts SO. Physical activity for the chronically ill and spina bifida during the transition from pediatric to adult disabled. Sports Med. 2000;30:207\u2013219. care. Annual Symposium Proceedings\/AMIA Symposium. 2005:580. 286. Weiss J, Diamond T, Demark J, Lovald B. Involvement in Special Olympics and its relations to self-concept and 271. Tan MJ, Klimach VJ. Portfolio of health advice for young actual competency in participants with developmental people with disabilities transferring to adult care. Child disabilities. Res Dev Disabil. 2003;24:281\u2013305. Care Health Dev. 2004;30:291\u2013296.","16 The Assessment of Human Gait, Motion, and Motor Function James J. Carollo and Dennis J. Matthews Instrumented gait analysis has evolved into a recog- deficits during the walking cycle. Fundamental to nized objective evaluation that is important in surgical making this connection is a clear understanding of and rehabilitation therapy planning for the child with the functional demands of normal gait. Recognizing an abnormal walking pattern. The technology related the essential features of normal, efficient locomotion to gait and motion analysis has improved significantly provides the basis for identifying the absence of these in recent years, enabling the collection and analysis of features in the child with gait dysfunction and, when large amounts of data obtained simultaneously from a applied systematically, can provide a strategy for clin- variety of specialized measurement instruments. The ical gait analysis (2). resulting quantitative description provides a compre- hensive snapshot of the subject\u2019s movement pattern at Therefore, the goal of this chapter is to familiar- a particular point in their development or at discrete ize the clinician with basic gait analysis principles intervals in their treatment. The clinician can use by focusing on the inherent functional requirements this information to describe the complex physiologi- of normal locomotion. This provides a framework for cal interactions that lead to abnormal movement and using specific gait measurements to pinpoint the joint motor control, and better understand their impact on or muscle system responsible for a particular func- gait, movement, and other functional activities. tional deficit, which can then be the target of appro- priate clinical interventions. A clear understanding of instrumented gait ana- lysis data and the ability to perform a meaningful NORMAL GAIT IS CYCLICAL interpretation that is clinically relevant remains a chal- AND SYMMETRIC lenge for many physicians. This may be attributable to the specialized nature of the gait analysis report or The principal goal of locomotion is to propel the body the false perception that an extensive biomechanics forward as efficiently as possible. The most natural background is required to integrate movement data way to accomplish this task is to employ a bipedal gait into the clinical decision-making process (1). More pattern, where the base of support alternates from one frequently, however, the underutilization of modern leg to the other. Inman has described the cyclical alter- gait analysis techniques in pediatric rehabilitation is ation of each leg\u2019s support function and the existence related to the difficulty associating gait measurement of a transfer period when both feet are on the ground deviations seen in the report with specific functional","462 Pediatric Rehabilitation as essential features of normal locomotion (3). Since lasting approximately 10% to 12% of the gait cycle at normal gait assumes no biomechanical advantage pro- typical walking speeds. These are generally described vided by either limb, a natural consequence of these as initial and final double support, but can also be essential features is the existence of a repeatable pattern identified in the context of the leading limb as right that is both cyclical and symmetric. Figure 16.1 illus- or left double limb stance period. The duration of the trates one complete gait cycle, or stride, and includes double limb support periods decrease with increasing the time periods and temporal events associated with walking speed, reaching zero at the moment running foot\/floor contact that necessarily arise from chang- begins. The time interval between the initial and dou- ing the support limb. Temporal events are specific ble support periods is defined as the single support moments in time that divide the gait cycle into discrete period, and is the same duration as the swing period time periods of specific duration, and are identified by of the opposite limb. Assuming normal symmetry, any the stick figures along the top of Figure 16.1. Typically, reduction in double limb support time is absorbed by a a cycle begins when one foot makes contact with the proportional increase in single limb support time, but walking surface (initial contact) and ends when that since single limb support always corresponds to the same foot strikes again. This is the functional defini- contralateral swing period, the overall stance period tion of a stride. Using such a convention allows a stride decreases, reaching 50% at the initiation of running to be time-normalized, where a specific stride location when double limb support reaches zero. When both is expressed as a percentage of the total cycle time or limbs\u2019 primary temporal events of foot-strike (initial stride period. Time normalizing the gait cycle facili- contact) and foot-off (terminal contact) are repre- tates comparing subjects with different stride lengths, sented on the same time scale, the duration of each stride periods, and walking speeds on the same scale. time period is easily illustrated. These general terms Figure 16.1 illustrates the time periods and tempo- for temporal events are applicable, regardless of gait ral events relative to the shaded ipsilateral side. If a pathology. Other terms are routinely used to identify subject\u2019s gait pattern is normal, the stride would be temporal events marking the transition from swing cyclical and symmetric inherently, and so be equally period to stance period (heel strike, forefoot initial con- ascribed to either side. The temporal event of foot off tact, foot flat) and stance to swing (toe-off, push-off), (sometimes referred to as terminal contact) separates but should only be used when they clearly describe the the gait cycle into stance and swing periods. Typically, observed foot\/floor contact pattern. stance period accounts for 60% to 62% of the total gait cycle and swing period takes the remaining 40% to While period durations relative to a single side 38%. We have intentionally refrained from using the are easily described when the gait cycle is represented terms \u201cstance phase\u201d and \u201cswing phase\u201d here to avoid on a linear scale, left\/right symmetry may be more confusing these intervals with the phases of gait to easily conceptualized when the gait cycle is wrapped be introduced in a later section, although in common around a unit circle (2,4,5), as shown in Figure 16.2. practice, the terms can be used interchangeably. For typically developing children and adults, ipsilat- eral and contralateral initial contact and foot-off will Stance period includes two intervals of double occur directly opposite each other around the circle, or limb support at the stance\/swing transitions, each 180 degrees out of phase. This graphically illustrates Initial contact Opposite Opposite Foot-off Initial contact (Foot-strike) Foot-off (Foot-strike) Foot-strike (Terminal contact) Initial Double Stance Period Swing Period Support Single Final Support Double Support 0% 12% 50% 62% 100% Figure 16.1 A typical gait cycle normalized in time, and represented on a linear scale from 0\u2013100% of the total stride. This repeating cycle begins with initial contact and ends with the next initial contact of the same foot. The stick \ufb01gures shown on top represent temporal events associated with foot-to-\ufb02oor contact. They divide the cycle into swing and stance periods, one period of single support and two equal periods of double support.","Chapter 16 The Assessment of Human Gait, Motion, and Motor Function 463 Initial contact not synonymous. Step length is the distance (in the (Foot-strike) direction of progression) from a point of ground con- tact of the trailing foot to the next occurrence of the 100% 0% Opposite same point of ground contact with the leading foot. It Foot-off is measured during initial double support and named Initial 12% for the leading limb. In contrast, stride length is the Double distance from initial contact of one foot to the next Support initial contact of the same foot, corresponds directly to the stride period, and is equivalent to the sum of suc- (Opposite Swing Single cessive left and right step lengths. Recognizing that single Period Support speed is defined as the ratio of distance per unit time, step length, stride length, cadence (steps per minute), support) Stance and walking speed are mathematically related by Period simple formulae: Final (Opposite walking speed (m\/s) = (cadence \u00d7 stride length)\/120 Double swing or Support period) step length (m) = (walking speed \u00d7 60)\/cadence 62% These basic outcome measures of overall gait per- 50% formance, including the timing measures previously described and other quantities such a stance\/swing Foot-off ratio, are collectively known as temporal-distance or (Terminal contact) temporal-spatial parameters. They can provide con- siderable insight into the overall effect of subtle gait Opposite abnormalities on walking performance. For example, Initial contact children with cerebral palsy may experience foot clear- ance problems during limb advancement due to exces- Figure 16.2 A typical gait cycle normalized in time, but sive ankle plantar flexion or decreased knee flexion wrapped around a continuous unit circle to illustrate during swing period. Evidence of this could be found symmetric phase relationships of temporal events and time in prolonged single support times on the more normal periods. The beginning and end of the cycle occur at the or less involved side, and a reduced stance period, step 12 o\u2019clock position. The temporal events of initial contact length, and stance\/swing ratio on the more involved and foot-off for each leg are typically opposite each other side (6). If the source of the limb advancement prob- on the unit circle, and the single support period of one limb lem can be attributed solely to the excess plantar flex- is equal to the swing period of the opposite limb. ion, the simplest intervention would be to prescribe a solid or leaf-spring ankle foot orthotic (AFO) with that the resulting time periods must be of equal dura- a rigid plantar flexion stop to restrict excess plantar tion for left and right single support, initial and final flexion during swing. Evidence that this intervention double support, and left and right swing periods. improved gait performance could be found in more Any disruption in the natural sequence of temporal symmetric single limb support times and step lengths, events anywhere along the cycle as a result of physi- a more normal stance\/swing ratio, and a higher walk- cal impairment, weakness, or spasticity will result in ing speed. incorrect timing for the events that follow. This nec- essarily leads to a loss of symmetry that can be quan- While clinical motion laboratories routinely com- tified by comparing the timing of temporal events pare a patient\u2019s temporal-spatial measures to age- between sides. Changes in symmetry reflected in the matched normative values, caution should be used gait period durations is an index of gait pathology, and when interpreting these results. Temporal-spatial measuring this simple quantity can be quite useful for parameters of cadence and stride length are directly evaluating treatment performance over time. related to walking speed (7), and since humans rou- tinely walk at a variety of speeds, simple deviations Since the duration of the swing period and leg from reference values alone may not be indicative of length determine the distance covered by the swing- gait pathology. Rather, reduced values for these mea- ing limb, deviation from normal symmetry and tim- sures may simply reflect the need to adopt a speed ing will give rise to differences in step length on each appropriate to the terrain, the required task, or the side, and subsequently total distance traveled per gait size of the room (8). A person\u2019s natural gait is also cycle. By definition, step length and stride length are dependent on the environment, with studies showing","464 Pediatric Rehabilitation that subjects walk faster on a long walkway compared pathology. Gait is most variable in the toddler, but grad- to a short one, and typically walk faster in outdoor ually stabilizes as the child reaches adolescence (9). studies compared to indoor studies (9). This lack of Hausdorff and colleagues have shown that the coeffi- consensus regarding normal values supports the con- cient of variation for stride time in typically developing vention adopted by most clinical laboratories to com- 3\u20134-year-olds is approximately 6%, but decreases to 2% pare patient results to their own laboratory-collected in 11\u201314-year-olds (13). In the elderly, increased vari- references, where these environmental factors can be ability is associated with increased risk of falling, with consistent for all subjects. Nevertheless, while it is speed variability the single best predictor of falls (9). \u201cnormal\u201d to walk at a variety of speeds, it clearly is These examples provide further evidence of the impor- abnormal to walk asymmetrically, so side-to-side dif- tance of a cyclical and symmetric gait pattern and how ferences in temporal\/spatial measures within a partic- variations in symmetry and cycle times reflected in the ular patient should always be investigated. temporal-spatial parameters of gait may be associated with gait pathology. When comparing temporal-spatial parameters in children, even greater care must be exercised, since TYPICAL COMPONENTS OF AN several age-related differences arise from the close INSTRUMENTED GAIT ANALYSIS relationship of these measures to leg length and gait maturity (10). Sutherland has shown that in typi- The phrase instrumented gait analysis (IGA) is often cally developing children, heel-first initial contact, used to describe the application of computerized mea- sagittal plane knee flexion wave, reciprocal arm surement technology to clinical gait analysis for the swing, and an adult joint angle pattern are acquired purpose of enhancing the interpretive power of the prior to the development of mature temporal-spatial analysis beyond what can be discerned using obser- parameters (11). All of these adult gait characteristics vational and physical examination methods alone. arise before the age of 3 years in most children (6). The specialized nature of the systems used to per- Because of this, Sutherland believes that gait matu- form an IGA typically requires a dedicated motion rity is best judged by the following five features, laboratory with specialists from clinical and tech- which he calls \u201cdeterminants of mature gait (11).\u201d nical disciplines to guide the patient through the These are: duration of single support, walking speed, testing procedures, make the required physical and cadence, step length, and ratio of pelvic span to ankle anthropometric measurements, and record and pro- spread (P\/A ratio). Notice that in addition to the first cess all data (Fig. 16.3). Analyses typically require four measures that are fundamental temporal-spatial 2 hours of patient contact time and between 8 and parameters, an anthropometric measure (P\/A ratio) 12 hours of processing and analysis time, depend- has been added, mainly to address the increased hip ing on the complexity of the patient referral and the adduction common in the immature child\u2019s gait. In number of measurements required to answer the general, walking speed, step length, single support, clinical question. It is not within the scope of this and P\/A ratio increase linearly with advancing age, discussion to comprehensively describe the full set of with the greatest changes occurring during the first measurement tools available for clinical gait analysis four years of life (6). Cadence decreases significantly between the ages of 1 and 2 years, after which it grad- Figure 16.3 A motion laboratory clinical specialist works ually continues to decrease (10). By age 4, the inter- to place re\ufb02ective markers on a subject while the technical relationship between temporal\/distance measures is staff prepares to record data for processing. fixed, although stride length and walking speed con- tinue to increase with increasing leg length. Muscle phasic alterations in the early walkers are generally characterized by prolonged activation periods and subsequent longer periods of agonist\/antagonist co- contraction around the joints of the lower extremities (12), most likely caused by neurologic immaturity associated with incomplete myeliniation (6). Despite all these age-related differences, the fundamental elements of a repetitive gait cycle are in place at a very early age. For this reason, asymmetric temporal\/ spatial measures can be used as indicators of gait pathology in both children and adults. Because normal gait should be cyclical and sym- metric, the existence of even small amounts of step-to-step variability may be an indication of gait","Chapter 16 The Assessment of Human Gait, Motion, and Motor Function 465 in children. For this, the reader is referred to sev- velocities, and accelerations of body segments through- eral excellent descriptions that are widely available out the gait cycle. Generally expressed in terms of the (5,14,15,16,17,18,19,20). However, since it is important joint angles between each limb segment, these quan- for the discussions that follow, we will briefly intro- tities are most often described three-dimensionally duce the primary measures used, some tips for their using anatomical planes relative to the more prox- practical application, and give examples of typical imal segment, but also includes the global position recordings as a reference. of the pelvis (pelvic tilt, obliquity, and rotation) and foot (foot progression angle) relative to a fixed labo- In addition to the temporal-spatial parameters ratory coordinate system located in the middle of the described in the last section, the primary measure- walkway. Modern kinematic analysis systems use an ments comprising IGA are gait kinematics, kinetics, assortment of markers or targets that are attached to and dynamic electromyography (16). While there are the subject at strategic locations and can be tracked certainly additional areas of measurement and many by specialized cameras or electromagnetic detec- useful instruments that can be included in a compre- tors (Fig. 16.4). The kinematic measurement system hensive IGA, these three measurement categories are identifies the position of the targets from multiple commonly accepted as the minimum necessary for perspectives in three-dimensional space using a high clinical evaluation of the patient with gait dysfunc- sampling rate (\u2265100 Hz) as the subject walks through tion, and have been identified by the Commission for a calibrated measurement volume. This determines a Motion Laboratory Accreditation (CMLA) as required unique trajectory for each target, which can then be for laboratory accreditation (21). reconstructed by the computer utilizing a kinematic link-segment model to produce a three-dimensional Gait kinematics is a general term that refers to animation of the walking subject within the virtual measurement of the linear and angular displacements, environment of the computer display (Fig. 16.5). From this mathematical representation of the subject, kine- matic graphs and interactive reports can be produced to facilitate the clinical analysis of the child\u2019s gait pattern. Kinematic measurement systems rely heavily on motion-capture technology and specialized software that fortunately have found a major market in the video game and motion picture industry. This has had the positive effect of substantially lowering the startup cost of these systems in recent years, making the technology more available to the clinical commu- nity and improving the accuracy, precision, camera resolution, and processing speed. These advances have also increased the complexity of the kinematic Figure 16.4 Subject with re\ufb02ective markers or targets Figure 16.5 Three-dimensional animation of the walking placed at strategic anatomic locations walks through subject within the virtual environment of a computer a modern motion analysis laboratory. The location of display. the targets depends on the mathematical requirements of the limb-segment model used to calculate the kinematic values needed for analysis. This subject is using a full body model based on the modi\ufb01ed Helen Hayes marker set.","466 Pediatric Rehabilitation models that can be implemented, which offers the these kinematic graphs in more detail when discuss- promise of more comprehensive and anatomically ing critical events in a later section. correct descriptions of motion. However, it may also introduce new challenges since increased model While measurements of gait kinematics pro- complexity necessitates greater software complexity. vide a quantitative description of body segment and Furthermore, the requirement for model validation joint movement during walking, gait kinetics focus with each new software release necessitates regular on describing the forces that cause these movements laboratory procedural changes, and may introduce and the calculated quantities that arise when forces data discrepancies when patient results are compared and three-dimensional kinematics are combined into over time using different models. Recognizing these a mathematical model of the body. Since joint and potential technical concerns, gait kinematics repre- muscle forces cannot be measured directly from the sent an integral component of clinical movement ana- walking subject, the forces due to foot\/floor contact lysis and are essential for analyzing the child with gait are measured using a specialized instrument known dysfunction. Figure 16.6 shows a set of three-dimen- as a force platform embedded in the walkway. The sional kinematic graphs associated with a sample of force platform measures the vertical, fore-aft shear, typically developing 12\u201313-year-old subjects used as and medial-lateral shear components of the ground a normal reference in our laboratory. We will discuss reaction force (GRF), which is the force vector acting at the supporting surface that is equal and opposite Kinematics Barefoot Walking to the sum of all muscular, gravitational, and iner- tial forces generated by the body in motion. Since a 30 Pelvic Tilt Pelvic Obliquity Pelvic Rotation force platform measures the magnitude and direction Ant 30 30 of the GRF as a single resultant vector quantity, only deg one foot can be in contact with the platform at a time Post Up Protr for a valid measurement. In order to measure multiple \u221230 foot strikes from both feet, the subject either needs deg deg to walk multiple times across a single platform or the laboratory needs to include a force platform array with Down Retr multiple platforms in different orientations so several clean foot strikes from both sides can be recorded in \u221230 \u221230 as few a number of passes as possible. A larger force platform array reduces alterations of gait characteris- Hip Flex\/Ext Hip Ad\/Abduct Hip Rotation tics in children with neuromuscular diseases in sev- 70 30 30 eral ways. Installing multiple force platforms into the Flex walkway reduces the number of trials required and deg Add Int thus minimizes the risk of fatigue. Furthermore, hav- Ext ing multiple force platforms lessens the possibility of \u221220 deg deg \u201ctargeting,\u201d which will alter the subject\u2019s character- istic gait pattern. Figure 16.7 shows the large 10-plat- Abd Ext form array of 60 cm \u00d7 40 cm force platforms currently used in our laboratory, and illustrates how rotating the \u221230 \u221230 long axis of each platform sequentially 90 degrees can accommodate a wide variety of stride lengths and step Knee Flex\/ Knee Var\/ Distal Shank patterns for children and adults. Ext Valgus Rotation The direct measurement of the individual force 80 30 30 components and the vector sum of the GRFs has his- Flex Var Int torically been used to evaluate gait kinetics and facil- deg deg itate a more qualitative pre-\/postsurgical comparison. Ext Val deg The most useful clinical application of gait kinetics, \u221220 \u221230 Ext however, is when it is combined with GRF measure- \u221230 ment and a kinetic model of the lower extremities to calculate joint kinetics, specifically joint moments and Ankle Dorsi\/ Foot 30 Ankle powers (22). The most common way to accomplish 80 Plantar 30 Progression Rotation this is to apply an \u201cinverse dynamics\u201d model of the lower extremity using the anthropometric dimensions Dors Int Int of each segment (typically seven segments, including the pelvis and both thighs, shanks, and feet) and esti- deg deg deg mates of each segment\u2019s center of mass and inertial Plan Ext Ext \u221230 20 40 60 80 \u221230 20 40 60 80 \u221230 20 40 60 80 Percent Percent Percent Avg File 9 Figure 16.6 Normal three-dimensional kinematic graphs constructed using a sample of typically developing 12- to 13-year-old subjects. These data are used as a reference for comparing kinematic data from clinical subjects. The dark line is the average of all subjects and the gray band represents +\/\u20131 standard deviation.","Chapter 16 The Assessment of Human Gait, Motion, and Motor Function 467 Figure 16.7 Large 10-platform array of 60 cm by 40 cm joint is quite useful because the magnitude and sign of force platforms used in The Center for Gait and Movement the curve at any instance in the cycle can illustrate if Analysis at The Children\u2019s Hospital in Aurora, Colorado. one half of the agonist or antagonist pair is dominating The \u201chopscotch\u201d pattern of the platform array permits the at a specific point in the gait cycle. Net moment values recording of several individual foot-strikes from both feet can aid in clinical interpretation of gait by comparing in a single walking pass. For illustration purposes each them to reference values for typically developing chil- platform is shown without its protective \ufb02oor covering, dren and by observing changes in the values before which caused the platforms to blend into the surrounding and after treatment. In addition, net moment values are walkway when applied. helpful in understanding how a child may be compen- sating at a given joint for weakness or limited range of motion at an adjacent joint. Figure 16.8 shows the sagittal plane kinematics, sagittal plane joint moments, and total joint power for the hip, knee, and ankle from a sample of typically developing 12\u201313-year-old sub- jects that we use as a normal reference. Once the three-dimensional moments at each joint have been calculated, joint power at any time in the gait cycle is the product of the joint moment and the corresponding angular velocity (instantaneous slope of the joint angular displacement curve from kinemat- ics) at each percent interval of the gait cycle: joint power = joint moment \u00d7 joint angular velocity (P(t) = M(t) \u2022 \u03c9(t)) Kinematics and Kinetics: Sagittal quantities. The forces at each joint can then be solved Hip Flex\/Ext Knee Flex\/Ext Ankle Dorsi\/Plantar sequentially, starting from the GRF at the floor and 70 80 30 working proximally, using the linear and angular forms of Newton\u2019s 2nd Law: Flex Flex Dors Linear: force = mass \u00d7 acceleration (F = ma) deg deg deg Angular: joint moment = moment of inertia \u00d7 Ext Ext Plan angular acceleration (M = I\u03b1) \u221220 \u221220 \u221230 By convention, joint moments can be considered Hip Flex\/Ext Knee Flex\/Ext Ankle Dorsi\/Plantar either external or internal. External moments reflect Moment Moment Moment the forces acting on the body through the skeleton that arise from the GRF, and since they reflect an exter- 1.0 1.5 2.0 nal biomechanical load, are sometimes called demand moments. Internal moments describe the force gener- Ext Ext Plan ated by the muscles and ligaments acting on the skel- eton to balance the external moments, and because Nm\/kg Nm\/kg Nm\/kg they are counteracting an external load, are sometimes Flex Flex Dorsi called response moments. Aside from their different functional descriptions, external and internal moments \u22121.0 \u22121.0 \u22120.5 for the same joint are of equal magnitude and differ only in their mathematical sign. The joint moments Hip Power Knee Power Ankle Power described in a typical IGA report are internal moments, 2.0 2.0 5.0 but this should always be confirmed since the sign and direction of the curves will be reversed if they actually Gen Gen Gen describe external moments. Joint moments are vector W\/kg W\/kg W\/kg quantities that describe the net torque around each joint but do not provide the individual force contribu- Abs Abs Abs tion from each agonist\/antagonist pair or from individ- ual muscles. Nevertheless, the net moment around the \u22122.0 20 40 60 80 \u22122.0 20 40 60 80 \u22122.0 20 40 60 80 Percent Percent Percent Avg File 9 Figure 16.8 Graphs of sagittal plane kinematics, sagittal plane joint moments, and total joint power for the hip, knee and ankle constructed using a sample of typically developing 12- to 13-year-old subjects. These data are used as a reference for comparing kinetic data from clinical subjects. The dark line is the average of all subjects and the gray band represents \u00b1 standard deviation.","468 Pediatric Rehabilitation Just as with joint moments, joint power reflects requires a bipolar arrangement of electrodes and min- the net power at a joint and not the individual power iature differential amplifiers with high common mode generated by a particular muscle or agonist\/antagonist rejection ratio (CMRR) placed close to the site of the pair. However, unlike kinematics and joint moments recording to ensure the EMG signal isn\u2019t overwhelmed that simply quantify the motion at a particular instant by motion artifact while the subject moves (Fig. 16.9). (kinematics) or calculate an estimate of the force Differential amplifiers with high CMRR (typically dominating the joint related to muscle function (joint greater than 100) amplify voltage differences between moments), joint power provides insight into the bio- the inputs and reject common voltages that may arise mechanical mechanisms responsible for specific move- from movement of the electrodes or the soft tissue vibra- ments and, in a sense, quantifies the actual \u201cmotors\u201d tion that occurs with foot contact. Surface electrodes are driving a particular gait pattern. In this way, joint the most commonly used electrode type for recording power curves are extremely useful to identify when a d-EMG from the pediatric patient to avoid the emotional particular joint is generating power (positive indicates trauma and change in gait pattern that indwelling elec- concentric contraction) or absorbing power (negative trodes often cause. Typically, the active portion of each indicates eccentric contraction) to analyze the trans- electrode in the bipolar pair should be small and the pair fer of power or energy from one joint to another and should be placed as close together as possible along the for understanding how one joint can compensate for long axis of the muscle (\u22641 cm diameter, \u22642 cm separa- disability at an adjacent joint. It should be pointed out tion) to minimize the effect of crosstalk from surround- here that although joint power is perhaps the single ing muscles. Unfortunately, surface electrodes are only most informative biomechanical variable that can be suitable for recording muscle groups that are directly obtained from an IGA, it does have limitations (23). For subcutaneous; if there is a need to evaluate deeper mus- one thing, power is technically a single scalar quan- cles individually, fine-wire electrodes made of a bipo- tity describing all planes of a joint combined, unlike lar pair of 50-micron platinum wire must be inserted displacement, velocity, and joint moment, which are directly into the muscle of interest using a 25\u201328-gauge directional vector quantities with individual compo- needle. When required, this is the most invasive aspect nent values for each anatomical plane. While in most of an IGA, and should be used only when necessary in cases the greatest contribution can be assumed to arise the pediatric patient and after all other data have been from the sagittal plane, the lack of a true directional collected, since the level of patient cooperation and the component (especially at the hip) may lead to incom- likelihood of a typical gait pattern decrease considerably plete clinical interpretations. Another issue is that since after a needle stick. In practice, most of the muscles of extensive use of mathematical modeling is required to interest to the pediatric physiatrist can be successfully arrive at the joint power values, there are numerous assumptions made in the process and great opportu- Figure 16.9 Patient with bipolar surface electrodes nity for errors or artifacts to influence the final curves. and small instrumentation ampli\ufb01ers for recording These issues should be considered when utilizing any dynamic EMG while the subject walks. This illustrates the kinetic variable for clinical decision making. However, electrode placement for the left vastus lateralis (distal they should not hinder the use of this information since location) and left rectus femoris (proximal location). Each these estimates cannot be obtained in vivo by any other electrode is connected to an instrumented backpack and means and still provide considerable insight into the then hardwired to the recording instruments. A wireless functional causes of gait abnormalities. EMG recording system using similar electrodes but with individual transmitters for each muscle is shown in Electromyography is an important tool for eval- Figure 16.4. uation of muscle and neurologic function and is well understood by the pediatric physiatrist. When used in the context of IGA, the purpose is slightly different from the conventional application. The primary objective of EMG in clinical gait analysis is to identify periods of muscle activation during walking so that decisions can be made regarding the appropriateness of muscle timing for agonists pairs as they selectively activate and deacti- vate during the gait cycle. This is the reason that we refer to this as dynamic electromyography or d-EMG, since the focus is on the phasic response of muscle during walking or some other functional activity. Since the subject won\u2019t be in a stationary position for the test, the instruments and technical procedures are also different from con- ventional diagnostic EMG. Dynamic electromyography","Chapter 16 The Assessment of Human Gait, Motion, and Motor Function 469 recorded using the surface electrode approach if proper to a normal EMG reference, and deviations from nor- procedures to minimize crosstalk and reduce motion mal are scrutinized for their contribution to the overall artifact are followed. movement pattern. Figure 16.11 shows filtered and time- normalized EMG for 12 muscles of the lower extremity Before the EMG recording can be used for clinical from a 15-year-old typically developing subject used as a interpretation, the raw data must be filtered, processed, laboratory reference, along with published normal EMG and time normalized so periods of muscle activation dur- activations represented as solid black bars at the bottom ing the gait cycle can be identified. A good reference for of each graph. The high-magnitude sections of the EMG processing guidelines is available from the International recording for each muscle correspond to the published Society of Electrophysiology and Kinesiology, where normal values, confirming that this typically developing they state that surface electrode recordings should be subject has a normal adult activation pattern. bandpass-filtered between 10 Hz\u2013350 Hz and fine-wire recordings filtered between 10 Hz\u2013450 Hz. This maxi- When EMG recordings are combined with the mizes the signal, minimizes the noise, and reduces kinematics, kinetics, temporal-spatial parameters, motion artifact. In modern systems, the filtered EMG radiographs, and the physical examination results, a data are sampled by analog-to-digital converters, and comprehensive snapshot of the subject\u2019s walking pat- further processing is performed by computer using spe- tern is revealed, providing an empirical basis for iden- cialized software or in concert with the motion-capture tifying the functional cause of a gait abnormality. To system. Data can be presented as a continuous recording use these data successfully, however, we must return of \u201craw\u201d EMG, an ensemble average of several cycles to the normal gait cycle to understand the functional of EMG normalized to the gait cycle, or as linear enve- requirements for walking, since these requirements lopes reflecting the EMG magnitude throughout the gait are a natural consequence of subdividing the cycle on cycle after rectification and integration of the raw EMG the basis of function. signal. In our laboratory, we also have developed a sys- tem to superimpose the EMG signal over the observa- IMPAIRMENT IDENTIFICATION IS tional video recording of the walking subject to screen FACILITATED BY SUBDIVIDING for faulty EMG recording during the analysis and to bet- THE GAIT CYCLE ter understand the interaction between observed move- ment and muscle activation (see Fig. 16.10, right side). While a repetitive gait cycle arises from the alternat- Regardless of how these data are presented, the goal is to ing base of support found in all bipeds, the existence use the EMG recording to identify periods of abnormal of this cycle provides great opportunity for clinical and muscle activity and determine if this activity is respon- biomechanical analysis of a child with gait dysfunc- sible for abnormal movement patterns presented by the tion. In particular, a repetitive cycle lends itself to nat- patient. Typically, the patient\u2019s activity is compared ural subdivision, which in turn, leads to a sequence of events that must be performed in order and with the Figure 16.10 Biplane high-de\ufb01nition video recording correct timing for efficient walking to occur. The earlier with superimposed real-time EMG traces of six muscles section titled \u201cNormal Gait Is Cyclical and Symmetric\u201d bilaterally from a typically developing subject used as discussed temporal subdivisions of the gait cycle delin- a reference at CGMA. The raw, un\ufb01ltered EMG recording eated by foot\/floor contact and their use in comparing provides immediate feedback on the quality of the EMG limb symmetry, measuring outcomes, and the general signal and the synchronization of muscle activity with characterization of overall gait performance. The focus observed movement. of the current section is to describe another type of gait cycle deconstruction, one based on functional subdivi- sions. For this approach, the functional prerequisites of walking are identified, and this provides a framework for subdividing the gait cycle into functional divisions (24). It is then possible to use the measurements avail- able from IGA to identify quantitative differences at each joint and the specific functional abnormalities that occur at critical moments in the gait cycle (25,26). Functional Prerequisites for Walking In their landmark paper published in 1953, Saunders, Inman, and Eberhart (27) described six gait subdivisions that they referred to as the \u201cdeterminants\u201d of normal","470 Pediatric Rehabilitation EMGS BF Walking 500 L Rectus Femoris 500 R Rectus Femoris \u03bcV \u03bcV \u2212500 L Vastus Lateralis \u2212500 R Vastus Lateralis 500 500 \u03bcV \u03bcV \u2212500 L Medial Hamstrings \u2212500 R Medial Hamstrings 500 500 \u03bcV \u03bcV \u2212500 L Anterior Tibialis \u2212500 R Anterior Tibialis 500 500 \u03bcV \u03bcV \u2212500 L Peroneals \u2212500 R Peroneals 500 500 \u03bcV \u03bcV \u2212500 L Triceps Surae \u2212500 R Triceps Surae 500 500 \u03bcV \u03bcV \u2212500 20 40 60 80 \u2212500 20 40 60 80 Percent Percent Left BF Avg EMG Control Right BF Figure 16.11 Filtered and time normalized EMG for 12 muscles of the lower extremity from a typically developing 15-year-old subject. The black bars at the bottom of each graph are constructed from published normal EMG activations and are used as reference values. The smooth curve above the EMG activation is a processed EMG signal obtained by rectifying and integrating the raw EMG. Notice that each EMG activation pattern above a baseline level is contained within the reference bar indicating a normal EMG pattern. In this typically developing subject, there is very little EMG activity from the rectus femoris during initial swing. gait. This treatise was significant in that it was per- the lower extremities and pelvis, each determinant haps the first formalized delineation of the gait cycle served to smooth different portions of the COM trajec- that explained how coordinated movements of the tory, effectively raising the COM during double support hip, knee, and ankle at specific points in the cycle led and lowering it during single support. While it is true to efficient forward progression. Each determinant\u2019s that unnecessarily large and abrupt movements of the influence on the three-dimensional path of the whole COM reduce gait efficiency, some of the specific deter- body center of mass (COM) was described using sim- minants identified by Inman and colleagues have now ple theoretical models, and the cumulative effect of been discredited (28,29,30). The improved accuracy and all six determinants led to a smooth, low-amplitude temporal resolution of kinematic measurement instru- trajectory that was assumed to be consistent with ments over the last 50 years have uncovered problems optimal, efficient locomotion. Specifically, Inman and with the timing of some of the theoretical mechanisms colleagues believed that minimizing vertical and hor- described in the original paper, and in the case of longer izontal motion of the COM would maximize walking step lengths, larger COM displacements are not neces- efficiency, since unnecessarily raising and lowering sarily associated with decreased gait efficiency. the COM would be wasteful from a potential energy perspective. By changing functional limb length with While the relevance of specific determinants may the addition of joints to an initially jointless model of now be in dispute, the real impact of this work is that it inspired generations of investigators to consider","Chapter 16 The Assessment of Human Gait, Motion, and Motor Function 471 biomechanical explanations for gait dysfunction and has the complete responsibility for supporting body led a few students of Dr. Inman\u2019s to develop clinically weight, maintaining whole-body stability (balance), applicable gait cycle decompositions derived from the and restraining forward momentum. This reduced functional requirements of walking. In a later mono- physical demand during the task of single limb sup- graph, Inman described two basic functional requisites port is due to the inherent passive stability provided of walking that he deemed necessary for any form of by the knee ligamentous structure and the force bal- bipedal gait, no matter how distorted by physical dis- ance at the hip as body weight moves forward (31). An ability or assisted by prosthetic or orthotic devices (3): essential functional requirement for this task is strong continuing ground reaction forces that support the eccentric contraction of the calf musculature to con- body and periodic forward movement of each foot trol the tibia (and subsequently the rest of the stance from one position of support to the next. limb) as it rotates over the fixed base of support pro- vided by the foot. When the task of single limb support These essential features of walking give rise ends and swing limb advancement begins, the physi- to a periodic gait cycle that must always be present cal demands increase once again, since the three goals for continued locomotion. An orthopedic resident of of weight transfer, limb advancement, and foot clear- Dr. Inman\u2019s, Jacquelin Perry, recognized that the phys- ance must all be accomplished. Similar to the weight ical demands of supporting the body against gravity acceptance task, important preparatory actions must varied, depending on whether the stance limb was begin before the swinging limb is lifted from the sup- accepting the initial impact or continuing to carry the porting surface at the end of stance period to meet the weight of the body during single support. To address functional demands of swing limb advancement. this, she developed the notion of three functional gait tasks (31): weight acceptance, single limb support, and Findings from other investigators support the exis- swing limb advancement. Dr. Perry considers weight tence of these three fundamental gait tasks, although acceptance the most demanding of the three functional each investigator has used a somewhat different termi- gait tasks since it requires the stance limb\u2019s muscula- nology when describing them (Table 16.1). Winter has ture and bony and ligamentous structure to provide characterized walking as an extremely complex motor shock absorption, initial limb stability (stiffness), and control task that requires three elements: support con- maintenance of forward progression. Preparation for trol to prevent collapse against gravity (32); balance the demands of weight acceptance begin late in swing control of the head, arms, and trunk (HAT) acting as period, when prepositioning of the leading limb occurs an inverted pendulum (33); and safe and coordinated to correctly align the foot to accept weight at initial lower limb movement during swing for minimum foot contact. The physical demands are lower for the task of clearance and gentle heel contact (19). Dr. Winter and single limb support, despite the fact that one leg alone colleagues have also stated that the goals of these tasks 16.1 Functional Subdivisions of the Gait Cycle Attributed to Different Investigators Investigator Inman Perry Winter Gage Subdivision Requisites Tasks Motor Control Tasks Prerequisites nomenclature Weight acceptance Functional Continuing ground reaction Support control to prevent Stability of the weight subdivisions forces that support the Single limb support collapse against gravity bearing foot throughout body Swing limb stance advancement Balance control of the HAT Periodic forward movement Clearance of the non\u2013 of each foot from one Safe and coordinated limb weight-bearing foot position of support to movement during swing to during swing the next achieve Appropriate prepositioning \u25a0 Minimum foot clearance of the swinging foot \u25a0 Gentle heel contact in preparation for initial contact Adequate step length Energy conservation HAT, head, arms, and trunk. Source: Refs. 3, 19, 31\u201333, 35.","472 Pediatric Rehabilitation can still be accomplished after disease, injury, or loss of use the term \u201cphase\u201d for intervals that have specific function because of the inherent redundancy of lower functional significance and have a clear relationship to extremity musculature and rapid adaptability of the cen- the three identified gait tasks described in the previous tral nervous system (34). It is interesting that Perry and section. The Rancho classification provides a framework Winter have identified essentially the same three gait for functionally organizing the gait cycle harmoniously tasks, despite approaching the study of gait from two with the three fundamental gait tasks, and after 30 years different perspectives: clinical analysis of pathologic of refinement, this approach has proven to be a power- gait and biomechanics of human movement, respec- ful tool for identifying specific functional deficits or gait tively. This lends support to the existence of these three impairments during each phase of gait. Instrumented elements and warrants using them to understand func- gait analysis can be used to quantify the magnitude of tional deficits in subjects with gait pathology. a functional deficit at a joint by reviewing the kinematic and kinetic data, or abnormal muscle timing by review- Gage has expanded on this description by identi- ing the EMG. This provides evidence and helps pinpoint fying five elements essential to walking that he has the specific region or system most responsible for the referred to as \u201cpriorities\u201d or \u201cprerequisites\u201d of normal overall gait abnormality, and suggests interventions to gait (35). This functional subdivision of the gait cycle directly correct the identified functional deficit or gait encompasses the three tasks described previously, impairment in each phase. but adds swing period elements necessary to ensure appropriate weight acceptance and the global task of The eight phases described by Dr. Perry are iden- energy conservation. In the order of functional priority, tified in Table 16.2. Notice that all but the first phase these are stability of the weight bearing foot through- (initial contact) represent separate time intervals out stance, clearance of the non-weight bearing foot between 0% and 100% of the gait cycle. Figure 16.12 during swing, appropriate prepositioning of the swing- illustrates the phases of gait in sequence around the ing foot in preparation for initial contact, adequate step unit circle, with stick figures signifying the temporal length, and energy conservation. This prioritization is influenced by Dr. Gage\u2019s interest in the gait of children 16.2 The Eight Phases of Gait as with cerebral palsy, and includes a gait efficiency task Described by Dr. Jacquelin (energy conservation) to address the reduced func- Perry tional capacity or endurance of many individuals with pathologic gait. He also identifies several physiologic PHASE OF DESCRIPTION and biomechanical mechanisms common to normal GAIT gait that can improve energy conservation. These are eccentric muscle contraction, return of \u201cstretch energy\u201d Initial contact The moment when the foot strikes the from prestretched muscles immediately prior to concen- Loading response ground tric contraction, bi-articulate muscles functioning as Mid-stance energy transfer straps, and joint passive stability from Initial double support period when the the effects of ground reaction forces whenever possible Terminal stance limb is accepting weight. to spare muscle activation (36). While other investiga- Pre-swing tors have addressed the functional prerequisites of gait First phase of single support when the (15,25,37), the contributions described previously form Initial swing body advances over the stance limb the basis of the strategy described in this chapter. Mid-swing ending ahead of the stance limb as Terminal swing weight is transferred to the forefoot Phases of the Gait Cycle Last phase of single support ending with Since the tasks of weight acceptance, single limb support, opposite initial contact and swing limb advancement can only be accomplished successfully if appropriate limb movement patterns occur Final double support period when the sequentially and with correct timing, Dr. Perry devel- knee rapidly flexes in preparation oped a systematic method of subdividing the gait cycle for swing and weight is shifted to the to simplify pattern identification and facilitate observa- opposite limb tional gait analysis (24). Now known as the Rancho clas- sification in honor of Rancho Los Amigos Medical Center 1st third of swing period where maximum where Dr. Perry and colleagues of the Pathokinesiology knee flexion occurs Service developed this method, it relies on eight subdivi- sions of the gait cycle, referred to as phases of gait. While Middle third of swing period where in general, both phases and periods refer to specific time maximum hip flexion occurs, ending slices around the gait-cycle unit circle, Perry prefers to with a vertical tibia Last third of swing period where final knee extension achieves maximum step length and the limb is properly positioned for weight acceptance"]
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