Improved medical care, increased life expectancy, and better services for lifelong care and support in society have provoked an interest and need for long-term future planning. This includes transitions in care from typical nurturing pediatric care systems to more traditional adult self-directed services. Although the notion of transition of youth with chronic conditions and disabilities is not new, the complexity of transitions, the possible negative outcomes in health, and the need for understanding aging with disability are now being recognized (25). Retrospective reviews and anecdotal experiences also question some long-held beliefs of “use it or lose it” to one of “conserve it to preserve it (26).” Choice of health care providers for adults with early-onset disability and special health care needs is, unfortunately, often limited by insurance and expertise.

Clinicians with an understanding of the natural history of disabling conditions can be helpful in monitoring, keeping vigilance for, and prevention of some general health conditions and aging or secondary conditions seen in disability. This public health model of prevention also includes tertiary prevention with the use of environmental modifications and technologies and removal of barriers to participation. There are general aging, associated conditions, secondary conditions, and health concepts that are helpful in understanding a life-span perspective.

The health of aging people with childhood-onset conditions can be seen as a dynamic balance between opportunities and limitations, shifting through life (32). Health perception is an individual determination, and is affected by personal expectations, experiences, sense of vulnerability, support, and locale. How people with disability self-rate their health has been questioned (33). This self-concept may also direct consideration of engagement in typical health and wellness activities. Often, the health of persons with disability is perceived as poor by clinicians and providers when individuals report a positive perception of their own health. This health provider concept may limit the offer of screening or health promotion opportunities. Perception of health in adults with disability may be related to time of onset; it is reported that adults with early-onset disability may identify better health than those with adult-onset disability (34,35). Research further suggests that adults with disability likely have a different construct of and self-rating process for health (36). Therefore, there has been growing interest in concepts associated with QOL.

QOL is a multidimensional construct reflecting subjective perceptions of the individual’s position in life in the context of the culture and value systems in which he or she lives and in relation to the individual’s goals, expectations, and concerns (37). QOL refers to the notion of holistic well-being, whereas health-related quality of life (HRQOL) specifically focuses on health-related aspects of well-being (37). HRQOL includes elements about physical and mental functioning, as well as the person’s appraisal of their effect on daily life and social functioning. For individuals with disability, HRQOL may be restricted. In general, persons with nonprogressive disabilities should be considered healthy, with a shift of the health care model from an illness and disability paradigm to one of wellness and prevention or early identification of secondary conditions, aging issues, and/or comorbidities.

GENERAL KNOWLEDGE REGARDING HEALTH AND PERFORMANCE

A body of literature has accumulated regarding health, aging, and secondary conditions for adults with disabilities and for some specific disabilities of early onset. Most research has focused on disabilities and impairments that have relative higher prevalence rates (eg, CP, ASD, speech–language disorders); are easy to associate with a disability group (eg, SB, Down syndrome [DS]); benefit from organized, dedicated service programs (eg, muscle disorders); and therefore have attracted research funding to generate a significant body of knowledge about the condition. The literature includes a combination of scientifically observed and anecdotal information as the database, often involving a “convenience” sample and a cross-sectional approach. Conclusions are drawn from patient reports, clinical observations, and ICD codes. More recently, standardized surveys or severity measures are used, and these provide a glimpse into individual characteristics or outcomes. Most studies identify health issues or concerns, with few challenging prevention or intervention strategies. Each factor in the interaction of disability and aging or secondary conditions has the capability to become a “negative feedback loop” (38) that may lead to further disability or a new health condition. There are studies using cross-disability groups that may have a higher representation of certain disability groups or may be small sample sizes, and consequently generalization to other disability groups should be considered with caution. In a similar manner, prevalence rates for some aging, secondary, and health conditions in disability-specific studies cannot be applied to all disability groups.

There are anticipated health and performance changes with aging. The risk for additional health problems should be monitored and addressed as with the general population. However, people with disability are often not afforded typical screening as in the general population. Iezzoni et al reported those with mobility impairments did receive pneumonia and flu vaccines, but were less likely to receive other preventive services. Women with severe mobility impairments in particular were less likely to receive Pap smears and mammography screenings (40). Cryptorchidism in males may be missed if examination is dependent on standing for examination. Women with disabilities had less knowledge about cardiovascular risks and no screening for risk factors, despite their higher risk with low activity levels (41). However, Cooper described a minor modification in office-screening techniques for adults with intellectual disability (ID) that improved the identification of risk factors and health needs, with improved health determinants (42). Lennox has shown that a Comprehensive Health Assessment Program (CHAP) resulted in increased health promotion, disease prevention, and case-finding in both community living and supported living adults with ID (43). Additional health risks and conditions can affect general performance.

Performance changes with aging include decrease in strength, balance, flexibility, coordination, and cardiopulmonary function, to name a few. The impact of these known aging changes on a person with mobility impairment is not well understood. Use of equipment, modifications to environment or activities, and joint protection all contribute to maintaining function over time. It is, however, known that persons with mobility impairments use more energy to perform mobility activities than their nondisabled peers. Therefore, exercise and activity to improve performance and maintain those improvements would seem intuitively obvious. In fact, there is scientific evidence that exercise and activities are effective for people with mobility impairments and that these activities can be managed through home programs and health clubs or aquatic activities, not just traditional physical therapy programs (44,45). Simply continuing typical activities, even though considered “strenuous,” will not increase strength, conditioning, or performance. Exercise and activities should be a part of a health maintenance program for adults with mobility impairments.

Participation (eg, social engagement, life role, employment) is one of the constituent elements of health and is increasingly considered a fundamental rehabilitation and health promotion outcome for child-onset disability. This is an important aspect of health and performance that should be queried when evaluating adults with pediatric-onset disability. We now have increasing generations of adults with childhood-onset disability who have the same aspirations and rights to participate fully in society. The literature on the unique needs and experiences of people with childhood-onset disability has taught us that we need to focus on physical, social, cognitive, and emotional aspects of health, with participation as one of the ultimate measures of outcome (ie, involvement in a life situation) (46). There is strong evidence that participation contributes to children’s physical, mental, and social well-being and to their QOL (47), and there is no reason to assume that this will be different when these children develop and live an adult life. Youth with physical disability, speech and language delay, or developmental disorders are at risk for lower community participation and social isolation, since they do not experience the widening social world of other teens (48). Tan et al (3) found that younger age, severe physical disability, and ID are important determinants of social participation in individuals with CP. Positive social experiences from early in childhood and continuing through adulthood prepare and support individuals with pediatric-onset disability to achieve positive sexual health and adult sexual roles (49). It is through these opportunities that people with pediatric-onset disability will develop friendships and romantic relationships. The results of the National Longitudinal Transition Survey Wave 2 showed that engagement in work or time with friends was most common for youth with learning disabilities or speech, visual, or other health impairments compared to other disabilities; more than three-fourths of youth in these categories were engaged in some type of employment including part-time jobs and unpaid volunteer work (50). In contrast, youth with ID (52%), multiple disabilities (54%), ASD (56%), and orthopedic impairment (59%) had the lowest rates of engagement.

Abbreviations: BIA, bioelectric impedance analysis; CIC, clean intermittent catheterization; DXA, dual energy x-ray absorptiometry; ENT, Otolaryngologist; GERD, gastroesophageal reflux disease; UTI, urinary tract infection.

Health outcomes are also evaluated by the use of medical services. Despite reports of good health, a Canadian publication notes adults with CP visited outpatient physicians 1.9 times more than age-matched peers. Annual hospitalization admission rates were 10.6 times higher for adults with CP compared to their peers (66). The presence of other medical conditions is associated with increased odds of hospital or emergency department (ED) use (67). Analysis of the Canadian Institute for Health Information notes epilepsy and pneumonia are the top two reasons for hospital admissions for youth and young adults, and for young adults only, mental illness is the third most common admission diagnosis (68). Additional adult diagnoses included lower gastrointestinal (GI) problems or constipation, malnutrition or dehydration, upper GI problems, and two unique problems seen in the adult group: fractures and urinary tract infections (UTIs). These represent conditions for surveillance in an adult population of people with CP.

Functional Status and Performance

The functional status of adults with CP is not static over time, and with aging there can be modest decreasing function, as there is for the general population. A number of studies, both in the United States and abroad, with small to large convenient samples, have noted that about a third of subjects report modest to significant decreases in walking or self-care tasks (34,52,69–71). Changes in dressing and walking with relative sparing of other self-care or social activities were reported in two of these studies (34,52). More recent studies provide data by Gross Motor Function Classification System (GMFCS), which allows better definition. Day et al used the large California database to determine the probabilities of loss or gain of walking skills into adulthood for those with CP (72). They noted that by age 25, there would unlikely be any improvement in walking skill and most would not change over the next 15 years, although there could be some decline. Therefore, the reason for even modest decreasing skill is not clear and may be related to progressive neurologic problems (eg, cervical spine stenosis, radiculopathy), lack of environmental modifications, pain, no access to or participation in exercise or activity programs, aging, or other medical conditions.

It has been suggested through cross-sectional and convenience samples that adults with congenital or childhood-onset disability may show musculoskeletal, mental health, or performance changes typical of advanced aging earlier than their nondisabled peers (51,59,73) (7,74,75). These observations require confirmation through longitudinal controlled studies. While risk factors may predispose a person to these changes, they are, as yet, unproven. If these earlier-than-expected aging changes are confirmed, they should be considered secondary conditions.

Pain and Fatigue

Pain is the most consistent health condition reported by adults with CP (35,59,74,76,77). It has been reported in a number of samples of adults with CP at a variety of ages to be 30% to 80%, with activity limitation from this at greater than 50%. For this reason, it will be covered as a separate topic. Communication challenges and multiple pain etiologies complicate diagnosis and treatment. Pain in CP may be present for a variety of reasons; it may be acute, recurrent, or chronic (78). Increased spasticity, weakness, falls, or progression of contractures or deformities can result from pain, particularly when pain is not reported because of communication difficulties or severe ID. Because of the high prevalence, the health care provider should try to elicit complaints or indications of pain, and evaluation, diagnosis, and intervention should ensue (78). Pain is often the reason for a change in function, living arrangement, or social interaction.

Pain is usually identified by proximity to a joint, and less often a limb. Most people report “arthritis” as the etiology of these pain complaints; however, these pains may originate from either joints or muscles. A good history and clinical exam will help sort out the issues and direct appropriate treatment. Back, leg, and hip pain complaints are common in persons with CP (79,80). There are usually more pain complaints in those with spasticity (79). It has been reported that fatigue often incites pain, and exercise most commonly relieves pain (79,81).

Fatigue is a common complaint of adults with CP, and is associated with pain (74,82). It is also associated with deterioration of skills, depression, and low life satisfaction, with no association with any specific type or severity of CP. As noted, it may incite pain. The fatigue may also be associated with the reported coping strategies sometimes used for chronic pain by adults with CP (83). Sleep disruption should also be questioned since it is commonly seen with pain and fatigue. Anecdotally, the pain/fatigue complex appears to respond positively to directed pain management, good sleep hygiene, medications, and exercise.

Appropriate management includes early identification of the problem and its source. Common musculoskeletal etiologies include poor ergonomics and biomechanics in tasks (secondary to deformity or limited motor control (71)), underlying weakness and therefore overuse (84), hypertonia (85), and degenerative joint disease (86). Typical management strategies should be offered, and referral for additional interventional, orthopedic, or neurosurgical consultation should be considered. However, adults with CP tend to self-manage their pain complaints (87), and for those who seek medical care, the report is minimal improvement and few options offered (88).

Musculoskeletal and Neurologic Conditions

CONTRACTURES. Contractures are a common secondary condition and may develop in childhood/adolescence and progress over the life span. Many individuals may have had interventions, including multiple surgeries in their life. Their impact on functional status or general health care needs is variable. Increasing contractures, particularly when associated with pain or increased spasticity, may be an indication of progressing pathology. Aging changes include decreased flexibility, and the clinician must distinguish pathologic causes of increasing contracture through appropriate diagnosis.

OSTEOARTHRITIS. Because of the significant pain complaints that adults with CP report, it is often stated that there is an early onset of osteoarthritis. Conceptually, this has been explained by unusual and possibly increased forces on joints that may be malaligned and/or have deformity; these forces are associated with underlying weakness and poor selective motor control (59). In fact, health care providers often will make a presumed diagnosis of “arthritis” for pain complaints in adults with disability. Clinically, it is not surprising to find significant arthritic changes with radiographs of painful joints, and sometimes at young adult ages. However, the presence of early-onset arthritic changes has been documented by case reports, and studies that report arthritis among subjects base this information on self-report of arthritis or presence of pain. Often, the pain complaint is not evaluated fully, and may have an etiology in soft tissue injuries or problems and not degenerative changes within the joint. There may, in fact, be premature osteoarthritis, but it has not been documented definitively. Of importance is the recognition of pain, appropriate evaluation, and treatment.

KNEE PATHOLOGY. Knee contractures are common in those who do not walk and in those who walk with obvious knee flexion and crouch. Not all knee contractures are painful. Tone management may improve range, function, and pain. Patella alta may develop from adolescence onwards over time, and pain or chondromalacia may result. Joint laxity may also be present. Modalities, exercise, kinesiotaping, and other interventions may be helpful. There are advocates for patellar tendon advancement surgeries, with or without distal femoral extension osteotomies, in adolescents and young adults to improve pain and restore knee function in gait, confirmed on gait analysis (97).

FOOT OR ANKLE PAIN. Again from biomechanical factors, contractures and pain may develop. Typical interventions may assist including orthoses, but not all bracing or shoe inserts are helpful, and biomechanics must be taken into account. Plantar fasciitis with appropriate treatment should be considered.

SPINE PATHOLOGY. In people with CP, severe motor impairment is associated with scoliosis and other deformities (98,99). Scoliosis may progress during adulthood, and those at 50 degrees or greater at skeletal maturity may deteriorate more rapidly (100). Scoliosis can cause seating and pressure problems, impaired respiratory function, and pain (85,100), and may be associated with windswept hips and pressure sores (85). It has been reported that spinal fusion improves the QOL for those with CP (101).

Spinal stenosis must be ruled out whenever significant functional change is noted, particularly for change in or loss of walking skills, increased leg spasticity, change in bladder habits, neck pain, vague sensory changes, and (late) change in arm and hand function (102,103). A tethering effect on the spinal cord also may occur, resulting in cranial nerve changes. Some early reports noted a higher risk in those with an athetoid or dyskinetic component (104,105); however, more recent reports show these problems are present in spastic forms of CP as well. While it is generally held that stenosis is due to early spondylosis and compression, there may also be a predisposition to it in those with a congenitally narrow canal, especially at C4 to C5 (102,104). Diagnosis is made through imaging studies, while comparatively evoked potentials may also be helpful in determining neurologic function. Surgical decompression may prevent further, often catastrophic, loss of function, but does not ensure return of lost function, particularly in cases of long-standing compression with spinal cord atrophy. Recurrence at levels above or below surgical correction may be noted (106,107). Postoperative management planning should accommodate changes in functional capabilities and care needs. The presence of an athetoid movement component will affect postoperative spine stabilization and possibly head positioning and neck mobility. When no surgical intervention is undertaken, a frank discussion of possible respiratory compromise and the future need for ventilator assistance should be provided.

PERIPHERAL NEUROLOGIC COMPRESSION. Radiculopathies may be a cause for painful complaints, and appropriate evaluation and treatment should ensue. It is most important that treatment strategies are based on the person’s history of function, that there is effective input from that person or their care provider, and that practical outcome goals are identified. Although not as common as a musculoskeletal etiology, nerve entrapment is also a cause of pain. The most common nerves and areas of entrapment as reported by adults with CP are the same as those susceptible to compression in the nondisabled population: the median nerve at the carpal tunnel and the ulnar nerve in the hand distally and at the elbow. Compression points are often related to use of crutches, transfer techniques, propelling wheelchairs, or existing deformity. Work-related or positional activities may also cause entrapments, just as in the nondisabled population. There is no reported increased incidence in CP. All hand pains or sensation changes do not represent nerve entrapment. Often, these complaints are actually problems of repetitive motion or are position-related. While they may be ascribed to carpal tunnel syndrome, they often respond poorly to surgery (108). Appropriate testing (including electrodiagnostic testing) is necessary to determine their etiology. Where treatment options are similar for disabled and nondisabled adults, some modification of management will be required if functional independence is changed by or during treatment.

Additional Health Conditions

There are no comorbidities known to be associated with CP. As noted, general health is good. Little is reported about cardiovascular health outcomes or risk factors such as reduced habitual physical activity levels and/or increased sedentary time in adults with CP. A study of adults living in group homes from upstate New York notes increasing health conditions with age for adults with CP as would be expected: cardiovascular, respiratory, and hearing/vision (113); this has been replicated in Taiwan and Israel (114,115). Of interest is that in comparison to U.S. national norms, there are fewer cardiovascular risk factors than seen in the general population. Peterson et al postulate that it is possible that adults with CP age more quickly than their healthy peers, resulting in an earlier onset of cardiovascular disease (CVD) (75). Either this is a healthier population or, more likely, there has not been effective screening and monitoring. Longitudinal research is necessary to determine risk factors and causation of cardiovascular events in aging adults with CP. In looking more critically at this population, the severity of the CP was related to increasing health problems with aging more than the diagnosis of CP alone (116). Vision and hearing problems may have been present early, and as anticipated, there is an increase in vision and hearing problems with age (113).

Dental issues are reported for adults with CP (79). Medications, nutrition problems, poor dental hygiene, and difficulty with access to dental care all contribute to the ongoing problems into adulthood.

Previously known associated conditions persist into adulthood. Dysphagia continues, and monitoring is required. Constipation also persists, and adjustments to bowel programs may be needed, in particular in individuals with more severe CP (117). From the latter study in children we can learn laxative use is high but dosing is frequently inadequate to prevent symptoms. Gastroesophageal reflux is often reported, but has not been present at increased rates. Intestinal obstruction is reportedly common in CP, and in an upstate New York cohort living in group homes, adults with CP had an increased rate compared to other adults with developmental disability (DD) (116).

Urinary incontinence may also continue, and it must be ensured that there is no dyssynergia or overflow with retention (118). Rosasco et al reported adults with CP had a higher incidence of UTIs that was related more to severity than the presence of CP (116), compared to other adults with DD living in group homes in upstate New York. UTI has been found to be a common reason for hospital admission for adults with CP (68). Neurogenic bladders in adults with CP are only infrequently associated with upper tract pathology (119). Some women report that incontinence consistently occurs at a particular point of their menstrual cycle, often associated with increased spasticity (120). Urinary incontinence can be effectively addressed through well-established diagnostic and intervention approaches. There are no available data that assess the adverse impact of urinary incontinence on sexual functioning and social integration in CP, but anecdotal support for this association is abundant. In both men and women, urinary incontinence should be identified and addressed, regardless of age or other conditions.

Respiratory problems have been implicated as the cause of death early in life and in early adulthood. Use of vaccinations may be helpful, along with vigilance and monitoring. Respiratory problems may increase with progressive scoliosis, and aspiration from gastroesophageal reflux disease (GERD) or dysphagia must be recognized. Sleep disorders related to pulmonary problems should be considered with progressive scoliosis, especially with complaints of poor sleep, morning headache, or daytime sleepiness.

There has been suggestion that obesity is a problem in CP, and yet studies are inconclusive (75,121). In fact, a small study of adults with CP identified mean body fat percentages and body mass indexes (BMIs) were within normal range, although 40% had heights below the fifth percentile for age and gender. Fifty-five percent reported dysphagia (122). Measurement of the percentage of body fat, which is the mark of healthy weight, is not routinely done, and state-of-the-art techniques (eg, DXA, bioelectric impedance analysis) have not been standardized for this population.

Sexual Functioning

Men with CP also should receive information on sexual functioning, including information on contraception and protection. There have been no reported problems with fertility. Most young men with CP reported a sexual interest (fantasizing or seeking erotic material), experienced feelings of sexual arousal, although some young adults with CP, however, experienced anorgasmia (49). Men with CP have a higher incidence of cryptorchidism (53%) at the age of 21 years compared with the general population (126), but this problem can easily be missed when the testis cannot be visualized or palpated with the patient in the standing position.

Adults with CP have many questions, including the use of contraception, heredity, pregnancy, assistive aids, spasticity, and pain. They also reported to have worries about interaction with a partner, experiencing a deviant body image, shame about their body, reactions of their body, and how to stimulate their partner sexually (127).

The topic of sexuality does not come up easily at appointments with physicians. Young adults with CP request an active role for the physician or other health care professionals to openly communicate on sexuality and provide the necessary information (127). Topics specific to CP that should be discussed are the following (127):