Functional Outcomes and Life Satisfaction in Long-Term Survivors of Pediatric Sarcomas

Article Outline

• Abstract
• Methods
  • Description of Objective Measures and Their Domains of Assessment
    • Six-minute walk test: measures performance, functional limitation, and aerobic fitness
    • Manual muscle testing: impairment
    • Grip strength testing: functional limitation
    • ROM: impairment
    • Limb volume: impairment
    • AMPS: disability
  • Description of Subjective, Questionnaire-Based Instruments
    • Human Activity Profile: performance and functional limitation
    • Lsm: Qol
    • Medical Outcomes Study 36-Item Short-Form Health Survey: disability
    • Sickness Impact Profile: disability
    • Goldberg Scale of Vocational Development
    • Occupational Performance History Interview
  • Statistical Analysis
• Results
  • Objective Measures and Their Relations
  • Subjective Measures and Their Relationships
  • Vocational Assessment
• Discussion
  • Study Limitations
• Conclusions
• References
• Copyright

Abstract 

Gerber LH, Hoffman K, Chaudhry U, Augustine E, Parks R, Bernad M, Mackall C, Steinberg S, Mansky P. Functional outcomes and life satisfaction in long-term survivors of pediatric sarcomas.

Objectives

To describe the inter-relationships among impairments, performance, and disabilities in survivors of pediatric sarcoma and to identify measurements that profile survivors at risk for functional loss.

Design

Prospective, cross-sectional.

Setting

Research facility.

Participants

Thirty-two participants in National Cancer Institute clinical trials.

Interventions

Not applicable.

Main Outcome Measures

Range of motion (ROM), strength, limb volume, grip strength, walk velocity, Assessment of Motor and Process Skills (AMPS); Human Activity Profile (HAP), Sickness Impact Profile (SIP), standard form of the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36); and vocational attitudes and leisure satisfaction.

Results

Twenty of 30 survivors tested had moderate or severe loss of ROM; 13 of 31 tested had 90% or less of predicted walk velocity; all of whom had trunk or lower-extremity lesions. Women with decreased ROM (r=.50, P=.06) or strength (r=.74, P=.002) had slow gait velocity. Sixteen of 31 tested were more than 1 standard deviation below normal grip strength. Eighteen had increased limb volume. These 18 had low physical competence (SF-36) (r=−.70, P=.001) and high SIP scores (r=.73, P=.005). AMPS scores were lower than those of the matched normed sample (P<.001). HAP identified 15 of 30 who had moderately or severely reduced activity. Leisure satisfaction was higher in the subjects (P<.001). Eight reported cancer had negatively impacted work and 17 reported that it negatively impacted vocational plans.

Conclusions

Survivors with lower-extremity or truncal lesions and women with decreased ROM and strength likely have slow walk velocity, low exercise tolerance, and high risk for functional loss. They should be identified using ROM, strength, limb volume, and walk time measures.

Key Words: Pediatric, Rehabilitation, Sarcoma

THE PAST SEVERAL DECADES have been associated with increased longevity for children and adolescents with solid tumors.¹ In fact, up to 75% of children with localized sarcomas are likely to be long-term survivors of their cancers.¹

Recent data² have shown that many children survive cancer, but may be at increased risk for the development of cardiovascular disease, hormonal abnormalities, and secondary malignancies,³ which occur well after completion of tumor treatment and result from treatment during the early stages of development.⁴, ⁵ Survivors of childhood cancers have decreased aerobic capacity, abnormal hemodynamic response to exercise, and relatively higher resting heart rates compared with age-matched controls.⁶ This would likely influence exercise tolerance, a final common pathway of overall performance of several organ systems.

Long-term studies of survivors of pediatric tumors have reported the effect of these tumors on quality of life (QOL),⁷, ⁸, ⁹ the psychologic impact,¹⁰, ¹¹ and vocational outcomes.¹², ¹³ Educational achievement has also been reported, and shown to be limited when compared with siblings and matched controls.¹⁴ Several of these studies were based on reviews of data sets developed from surveys, rather than from patient contact. Many of the study subjects were treated in multiple centers with a variety of chemotherapeutic agents and varied approaches to radiation and surgical treatments for sarcoma, making generalizations from these data uncertain.

We report here results in a cohort of cancer patients who received medical, surgical, radiation, and rehabilitation treatment at Clinical Center, National Institutes of Health (NIH). All treatment was provided in 1 facility; a well-trained multidisciplinary team of rehabilitation specialists performed follow-up, thus assuring a relatively complete and reliable data set.

Impairments of the musculoskeletal system are most frequently reported.¹⁵, ¹⁶ Function, which is often subjective and self reported, or objectively measured performance, are less likely to be reported in this population.¹⁷ Function is an excellent predictor of good health and longevity regardless of the disease process.¹⁸, ¹⁹ Low levels of ambulation and weak grip strength are risk factors for mortality in several patient populations.²⁰, ²¹, ²², ²³ Measurement of these may yield important information about current health status and prognosis, and identify opportunities for interventions to improve function, to reduce or prevent disability, and adverse sequelae of treatment.

We designed this exploratory study to evaluate function and performance in adult survivors of childhood and adolescent sarcomas. We wanted to determine whether this group differed from unaffected controls, and if there were associations among impairments and measures of performance and disability.

Based on the outcomes of this study, we asked whether a practicing physician would be able to identify contributors to decreased mobility and daily routines by using standard examinations and questionnaires. We also asked whether a “profile” of a cancer survivor at risk for functional loss might emerge from the grouped data. For example, would those with a specific tumor site or other impairments have a high degree of functional loss, warranting early intervention or prevention of further loss?

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Methods 

There were 108 identified survivors of pediatric sarcoma who had been treated with one of several treatment protocols for sarcoma at the National Cancer Institute (NCI), NIH, who participated in this institutional review board approved protocol. Subjects received no remuneration for participating. Eligible subjects provided informed consent to participate in this study. They were in remission were without evidence of cancer recurrence for at least 24 months; and had not received chemotherapy, radiation therapy, cancer-related surgery, or immunotherapy within the prior 24 months. All patients were treated with multiagent chemotherapy. Chemotherapeutic agents included vincristine, cyclophosphamide, doxorubicin, actinomycin D, etoposide, and ifosfamide. Details of treatment protocols are available.²⁴ Data were retrieved from medical and research records available electronically and from hard copy.

All patients received a standard assessment with instruments administered by rehabilitation specialists trained to assess these variables. For example, the Assessment of Motor and Process Skills (AMPS) was administered by a trained and calibrated occupational therapist, the Leisure Satisfaction Measure (LSM) was administered by a recreational therapist, and a physical therapist assessed range of motion (ROM) and walk times.

Description of Objective Measures and Their Domains of Assessment 

Subjects were instructed to walk as quickly as possible on an indoor gymnasium floor for 6 minutes. Heart rate and blood pressure were measured before the walk, immediately postexercise, and after 2 minutes of recovery. The distance walked was recorded, the patient’s velocity was calculated and compared with the velocity of normed subjects of the same age and sex reported in an established reference database.²⁵, ²⁶

We used the Kendall 10-point manual muscle testing (MMT) method to assess the strength of the affected and unaffected limb pair.²⁷ MMT evaluation was grouped as: normal (4) if the score was 9 or 10; mildly impaired (3) if the score was 7 or 8; moderately impaired (2) if the score was 4 to 6; and severely impaired (1) if the score was 0 to 3. An MMT score was calculated for the distal and proximal muscle groups of each limb by averaging each muscle’s score.

Gross grip strength was measured with a hand dynamometer. The average of 3 grip strength measurements was compared to average grip strength performance of normed subjects of the same age and sex reported in an established reference database. Both left- and right-handed grip strength were measured.²⁸, ²⁹

Active ROM was measured using standard goniometric measurements of the affected extremity, and graded as follows: normal (4); mild decrease, less than 15% deficit (3); moderate decrease, 16% to 40% deficit (2); and severe decrease, more than 40% deficit (1).³⁰

Limb volume was determined with an optoelectronic scanner system (Perometer).^31a Sensors are located within a rectangular frame, which is moved over the length of the extremity. Hands and feet are not within the scanning range. Based on an instrument-software interface, volume is calculated from circumferential limb measures determined by the sensors, and ratios of volumes of affected and unaffected limbs were determined.

Subjects were observed performing 2 familiar activities of daily living (ADL) tasks (eg, preparing food, cleaning up in the kitchen) and evaluated on 20 ADL process skills and 16 ADL motor skills. Each skill was evaluated on a 4-point interval scale, ranging from markedly deficient (1) to competent (4). The ADL motor skills were rated based on observable goal-directed actions enacted while doing ADL tasks to move one’s self or the task objects. The ADL process skills were rated based on observable actions to logically sequence and select appropriate tools, and to be able to adapt performance when problems were encountered. These were measured in real time. The patients’ scores were compared with age-matched normative AMPS data from the international AMPS database.³²

Description of Subjective, Questionnaire-Based Instruments 

The Human Activity Profile (HAP) is a 94-item questionnaire that measures a subject’s activity level. The questions are scaled based on the amount of oxygen needed to perform. For example, the first question asks if a subject can arise from a chair (2−3 metabolic equivalents [METS]) and ends with asking whether they are currently jogging 4.8km (3 miles) in 30 minutes (7−8 METS). There is an option for subjects to indicate that they have never done this activity, in which case the question is voided. A maximum activity score and adjusted activity score were calculated according to the HAP manual’s instructions.³³ The adjusted activity score is a measure of usual daily activities, and is the best estimate of the respondent’s average level of energy expenditure in comparison with peers of the same age and sex. The maximum activity score is the highest oxygen-demanding activity that the respondent still performs, and is the best estimate of the respondent’s highest level of energy expenditure, again in comparison with peers of the same age and sex. Subjects can often achieve this occasionally rather than routinely. Scores are obtained and ranked as follows: greater than 83 is normal; 73 to 83 is mildly disabled; 62 to 72 is moderately disabled; and less than 62 is severely disabled, as compared with other disability measures. The measure was self-administered; 30 subjects completed the activity questions.³³

The LSM³⁴ measures the degree to which a person perceives how his/her leisure needs are being met. Fifty-one statements about leisure activities, such as “my leisure activities help me to relax,” were presented to the subjects. They rated the statements on an ordinal scale ranging from “never true” (1) to “always true” (5) to obtain an overall score of leisure satisfaction. Psychologic, educational, social, relaxation, physiologic, and aesthetic subscores were calculated according to the LSM manual’s instructions. All 32 subjects completed the measure. Subjects were compared with a population of 347 students aged 20 to 25 years of age.³⁵

The Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36), version 2, evaluates general health status, with an emphasis on physical, social, and emotional functioning. The 36-item questionnaire was self-administered. Norm-based scoring was performed according to published methods, using SAS^b to produce a physical health summary measure, a mental health summary measure, and 10 subscales.³⁶ Subjects were compared to age- and sex-matched normed subjects from an existing reference database.³⁷ Thirty subjects completed the survey.

The Sickness Impact Profile (SIP)³⁸ measures perceived health status and assesses the impact of health on activities and behavior. The 136-item questionnaire describes everyday activities, such as “I do not keep my attention on any activity.” The measure was self-administered, with respondents marking those statements with which they agreed. Questionnaires were scored according to the SIP manual to produce 12 dysfunction scales and 2 higher-order dimensions summarizing physical and psychosocial domains. Twenty-two subjects completed the entire SIP. Another 8 patients completed only the mobility, attention, emotional behavior, and relaxation dysfunction scales because of an error in copying the questionnaire.

A vocational counselor conducted a semi-structured interview that included questions on pre- and post-illness educational and vocational plans and aspirations. Data were recorded verbatim. Two raters used standard coding to score and average the responses. A score of 0 to 4 was assigned for each category. A 0 score was given when there was no specificity, realism, awareness, or commitment demonstrated. A 4 was given when the greatest amount was demonstrated. Summing the individual categories produced a total score. Adults and adolescents were scored on different forms. A score less than 2.5 is below average. Six subjects were scored on the adolescent form of the Goldberg Scale of Vocational Development (GSVD)³⁹ specific for subjects younger than 18 years. Twenty-six were scored on the adult form.

An occupational therapist administered the Occupational Performance History Interview⁴⁰ to gather information on the subject’s occupational life history. The information was assigned a rating as an indication of the subject’s level of functioning on 3 rating scales: occupational identity, occupational competence, and occupational behavior settings. Occupational identity measures the degree to which a person has internalized a positive occupational identity (eg, having values and having an image of the kind of life one wants). Occupational competence measures the degree to which a person is able to sustain a pattern of occupational behavior that is productive and satisfying. Occupational behavior settings measures the impact of the environment on the person’s occupational life.⁴⁰

Statistical Analysis 

Statistical analyses were performed to assess descriptively the relationships among patient characteristics, impairments, functional limitations and performance, disability, and vocational and occupational behaviors. Analyses were performed using nonparametric statistical methods, such as the Wilcoxon rank-sum test for comparing 2 groups.

Many of the analyses were performed relative to published data from samples of normed subjects who underwent the same form of testing. To perform these comparative analyses, standardization was done, patient by patient, by subtracting the mean and dividing by the standard deviation (SD) of the best age- and sex-matched comparison group available from the result for each patient in this study. In some instances, the reference group was only that reported from a single published group of subjects, while, in other instances, individual age and sex cohort data were available. The corresponding matched age and sex result was used to standardize each patient’s score for the appropriate test. Each of these comparison groups is referenced in the above sections that described objective measures and questionnaires. Then, the resulting standardized value, which would be centered on zero if the groups were similar, was evaluated for its statistical significance by a Wilcoxon signed-rank test. A small P value would indicate a potentially significant difference from the reference sample, while a larger P value would indicate greater similarity between the 2.

Correlation analyses were done using the Spearman rank correlation. The correlation coefficients, denoted as |r|, are interpreted as follows: |r| greater than .70 as strong correlations, |r| range between 0.5 and 0.7 as moderately strong, and |r| range between 0.3 and 0.5 as weak to moderately strong.

The results presented are those that were found to be interesting clinically after a very extensive exploratory analysis, in which most analyses performed were not presented because of their lack of associations. In view of the many analyses performed, and their varying degrees of independence from one another, any formal correction procedure would not be readily interpretable. Instead, P values less than .005 are interpreted as being associated with statistically significant results, while those between .005 and .05 are associated with trends. As this is a descriptive analysis, there is no set threshold for inclusion of results that were of interest. P values are 2-tailed and not adjusted for multiple comparisons.

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Results 

We retrieved information that enabled us to contact 80 potential participants. Thirty-two agreed to participate, 19 declined, and 29 did not respond. Review of demographic information about the participants and nonparticipants showed no significant differences between the 2 groups in tumor type, age at diagnosis, location of tumor, presence of metastases, or radiation (table 1).

Table 1. Patient and Treatment Descriptors

Age (y)	No.
At time of treatment
6–10	4
11–15	14
16–20	12
>20	2
Mean age	16.2±5.2
At time of study
16–20	2
21–30	11
31–40	8
41–50	9
>50	2
Mean	35.4±10.6
Sex (male/female)	17/15
Tumor type
Ewing’s sarcoma	24
Alveolar rhabdomyosarcoma	3
Neuroectodermal	3
Sarcoma	2
Treatment
Surgical resection	18
Local radiation	26
Cranial radiation	8
Pulmonary radiation	6
Total body irradiation	8

Objective Measures and Their Relations 

Subjects had primary tumor sites from head to toe. Impairments and performance organized by tumor location are described in table 2. Four subjects were unable to perform the walk and run test because of lower-extremity pain, dysfunction, or inability to don prosthesis at the time of testing. They were included in the group that had less than 90% of predicted velocity for age and sex because of their inability to perform.

Table 2. Impairment and Performance Measures by Tumor Site

Abbreviation: NT, not tested.

Velocities tended to be slower for patients with tumors in the trunk, pelvis, and lower extremity, compared with those with tumors in the head or upper extremity (P=.023), and there was a negative correlation between velocity and age among all patients (r=−.38, P=.045). Also, among all patients, there was little difference in velocity between those with decreased ROM and those without (P=.38). Among those with ROM loss, walk times were substantially lower in patients whose lesion was in the pelvis or lower extremity, compared with those with lesions in other locations (P=.026). Girls/women had a positive correlation between velocity and either loss of ROM (r=.50, P=.06) or weakness (r=.74, P=.002).

There were differences between girls/women and boys/men. In girls/women, there was evidence of slower velocity when weakness was identified versus when it was not (P=.055); for boys, this difference was not present (P=.59). Additionally, boys/men and girls/women scored significantly differently in mental competence, as measured by the SF-36. Girls/women had higher standardized scores (P=.000). This was also true for the GSVD (P=.041), but to a lesser extent.

Eighteen patients had limb volume measures because of asymmetry of limb size. Those with higher limb volumes as measured by the Perometer had lower physical competence on the SF-36 (r=−.70, P=.001) and higher SIP summary scores (r=.73, P=.005). Those with greater swelling tended to be somewhat further out time-wise from their treatment (r=.49, P=.038) and were older (r=.46, P=.057). Interestingly, there was little correlation between the amount of swelling and dose of radiation (r=.33, P=.19).

Subjects had significantly lower AMPS motor and process scores than age-matched, normed controls (P<.001 by Wilcoxon signed-rank test on standardized scores). Fourteen (44%) had a raw motor score below 2.0 logits. A score below 2 logits on the motor scale and 1.0 on the process scale (cutoff ability measures) implies that a subject may require assistance or is at risk for safety in daily routines.

Subjective Measures and Their Relationships 

Data describing the level of performance based on the HAP showed that the adjusted activity scores and the maximum activity scores were moderately to severely reduced in 10 of 30 (33%) and 5 of 30 (16%), respectively. In addition, for maximum activity score, 20 of 30 (67%) patients scored below the 50th percentile for a matched sample of normed controls. By an exact binomial test, the P value associated with this was .099, suggesting limited evidence of a greater fraction below the 50th percentile than would be expected. For the adjusted activity score, 19 of 30 (63%) were below the 50th percentile (P=.20).

When SF-36 scores of the cohort were compared with a sample derived from normed subjects,³⁷ results showed a trend between the physical (P=.065) or the mental competency subscales (P=.058), with the physical competence scale tending to be lower than the norm, and the mental competence scale tending to be higher.

Results of LSM are presented in figure 1. The physiologic subscale, a measure of the relative satisfaction subjects received from activities designed to promote fitness, a healthy lifestyle, and to control weight, provided the least satisfaction. Relaxation provided the greatest satisfaction. After being standardized to a reference sample of 347 students aged 20 to 25, the scores were significantly higher than in the control group in overall leisure satisfaction and all subcategories (P<.001). A high standardized score is an indicator of greater leisure satisfaction.

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• Fig 1. 

  Leisure satisfaction measures in survivors of pediatric sarcoma. NOTE. Errors bars are SD. Legend: 1, extremely low satisfaction; 2, low satisfaction; 3, moderate satisfaction; 4, high satisfaction; 5 extremely high satisfaction.

Subjects had a mean SIP score of 6.2 out of 100 (range, 0−13.6). The general adult population has a SIP score of 5.³⁸ Higher scores indicate an increased perception of the impact of sickness. Overall, subjects perceived that their illnesses had a minimal impact. The range of scores was wide, however, suggesting some patients experienced a severe impact of illness on their life activities. Individually, subjects perceived a higher impact for some categories; the ranges are shown in figure 2. Of the tested categories, sickness had the greatest perceived impact on work for some subjects, with an average score of 19.2 (range, 0−70.1). Note that there was a bimodal distribution of the work SIP score, and the median score was 0.0.

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• Fig 2. 

  Sickness impact on survivors of pediatric sarcoma. SIP scores for 5 categories, 2 dimensions, and a total SIP score. Higher scores indicate perceived increased impact of sickness. Average scores are graphed.

Performance, health status, disability, and life satisfaction were determined from the self-reports and correlated with velocity (table 3). Performance measures (HAP and SIP ambulation subscale, AMPS motor) correlated significantly with velocity as measured by the 6-minute walk test (6MWT).

Table 3. Correlations Between Gait Velocity and Self-Reported Function and Performance

Abbreviations: AAS, adjusted activity score; MAS, maximum activity score; PCS, physical competence scale.

Vocational Assessment 

There are data on 6 adolescents and 26 adults from the administration of GSVD. Eight of 26 reported that they worked; 3 worked part-time and 5 full-time. More than 50% had deficits. Sixty-five percent (17/26) said their tumor had a negative impact on their vocational plans. Adolescents scored at or above means for age-matched controls except for their degree of occupational awareness (mean, 2.4). Adults scored below age-matched controls on predisability interests, and responsibility for vocational plans pre and post-disability. They scored high (4/5) on optimism about disability outcome. A score below 2.5 is considered a deficit and believed likely to require vocational counseling.

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Discussion 

Pediatric cancers affect only 1 in 300 to 350 people less than 20 years of age and the survival rate exceeds 75%.¹ The prevalence of cancer survivors 20 to 35 years of age is estimated at 1 in 500, a sizable group. This is a population that survives into early adulthood, but longevity may require management that is directed toward needs other than cancer treatment. These needs should include mitigation of the sequelae of treatment and its effect on cardiac function, limb swelling, strength, and motion, and physical performance, among others. Some of these have been discussed in recent publications.², ⁵, ²⁴ Based on the data reported here, functional loss is common in these cancer survivors and they should be evaluated and treated with a chronic disease management strategy aimed at risk identification and reduction of functional loss. Treatment targets should include maintaining or restoring good function because this will improve performance and likely increase longevity. We would not want to eradicate the cancer only to have patients succumb to premature and possibly preventable disease (eg, cardiovascular).

The noun “function” means an action or activity that is fitting or proper for a given organism or structure. It describes activities that support both necessary and desirable daily routines. Contributors to good function include adequate ROM, MMT, aerobic capacity, health status, motivation, cognitive ability, and self-image, among others. We chose to measure some of these that we know to be easily and frequently used, reliable, and correlated with functional outcomes.

The study reported here, although limited by a small sample size and based on a fraction (32/108) of the subjects who theoretically might have been possible to evaluate, identifies several differences between survivors of pediatric cancers and age-matched controls, based on this battery of assessments. Most notably, the objective measures ROM, MMT, grip strength, and 6MWT provide valuable information that may lead to interventions. Data suggest that in the elderly and nonelderly adult population, mobility²⁰, ²¹, ²², ²³ and grip strength²², ⁴¹ are reliable measures of, and correlate well, with longevity. Nineteen subjects had grip strength more than 1 SD below normal. It was surprising that only 3 of these subjects had upper-extremity lesions, and that the weakness was bilateral in 13. This suggests a more generalized problem of weakness or deconditioning than one attributable to a site-specific lesion. These subjects also showed lower performance on the motor portion of an objective measure of ADLs, and on a self-report of performance reported to correlate with oxygen consumption and aerobic capacity. The HAP correlates well with oxygen consumption measures and hence with gait velocity.³³

This study had a small sample size and therefore should be repeated, but with a larger sample. Such a study should be longitudinal and should assess the effect of fitness training, reducing limb volume, improving ROM and strength; and providing education about the importance of lifelong treatment aimed at reducing impairments, improving performance and function. Outcomes for such a study should include measures of performance, function and metabolic measures, all of which are likely to improve when risks are properly identified and treated.

Decreased gait velocity was likely to occur in subjects with lower-extremity lesions, moderate or severe abnormalities of ROM, and in women, with weakness. Other studies have found that tumor location is not a correlate of impairment⁴² or a predictor of function.⁴³ These studies differed from ours in that they used questionnaire based data exclusively and did not include truncal lesions when assessing mobility loss. Truncal lesions may have a negative impact on abdominal strength and balance, which can result in slower walk velocities.

We used a very sensitive, objective measure of ADLs, the AMPS, which showed that both motor and process scores were significantly below normal. This could not be explained on the basis of upper-extremity lesion location in that only 3 subjects had upper-extremity abnormalities, although all 3 had grip strength more than 1 SD below normal. Motor performance depends on stabilizing and positioning the body to support appropriate hand use. This may include, for example, bending, reaching, and rising up on the toes, any of which may be affected by weakness remote from the upper extremity.

Subjects also had lower process skill scores, reflecting difficulties or inefficiencies in initiating tasks, choosing correct ingredients, locating tools and ingredients in kitchen cabinets, organizing the work area safely and effectively, and producing the previously agreed on product. Some subjects, for example, demonstrated clear short-term memory problems during their tasks as they attempted to locate items shown to them in the kitchen immediately before starting the assessment. Beyond the direct demonstration of ability to organize a task and adapt when problems are encountered, the “AMPS process scale provides further information regarding the extent to which the person has overcome residual neuromuscular, biomechanical, cognitive, and psychosocial impairments and capacity limitations by using alternative or compensatory strategies during task performance.”^31(p4) For some subjects, generalized motor difficulty affects their overall ability to organize and adapt actions to complete a task.

Study Limitations 

We used self-report methods to identify what individual patients were doing, and to obtain this information from the patient’s perspective. Questionnaires have been shown to be reliable and sensitive to changes in performance. They are inexpensive, easy to use, and can be completed in a relatively short time.

There are shortcomings to using self-reports. One is that they often measure a combination of phenomena in the same instrument (eg, impairments, functional limitation, disability). Identification of major contributors to the correlations among our quantitative impairment and performance measures (eg, ROM, walk times, grip strength) and qualitative measures of function and QOL is difficult. This group of survivors, however, reported that they were moderately disabled (47%), similar to the number with impaired walk times (40%), which reinforces the view that patients’ perceptions about independent function correlate with this objective measure.

One of the most important developmental issues facing children and adolescents is prevocational experience and ultimate vocational plans and outcomes. Compared with normed adolescents and adults, predisability scores were below norms, but motivation and realistic interests postdisability were considerably higher than norms. This is consistent with other studies, in which cancer survivors engaged in less risky behavior with respect to drug and alcohol use⁴⁴ and showed responsible behavior with respect to follow-up health care, and were concerned about function, including marriage, education, occupation, and participation in peer-related activities.⁴⁵

We used the LSM as an indicator for non-ADL and avocational activities. It has been suggested that satisfaction with leisure activities is a good index of QOL. This group, as a whole, indicated a high level of satisfaction with leisure activities. The least satisfying activity reported involved physical activity; a finding that may contribute to the subjects’ poor performance in the 6MWT. The data we report here suggest something different from other reports,⁴⁶ which showed that physical sequelae are often less important contributors to QOL than psychologic and social ones.⁴⁷ Physical impairments in our group were significantly associated with limitations in both observed and self-perceived performance. Our subjects valued physical activity, and the loss of mobility was associated with a perceived negative impact on work, and greatest impact on feelings of sickness.

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Conclusions 

We believe there is a profile for pediatric sarcoma patients who are most likely to suffer poor functional outcome. The profile includes those with: (1) lower-extremity lesions (including pelvis and trunk), (2) limb edema, and (3) women with loss of ROM and weakness. Age is another risk factor for slow walk time, suggesting fitness should be a lifelong goal.

Women who have weakness and decreased ROM have decreased velocity, making them at greater risk than men for decreased mobility and poor aerobic capacity. If one combines the findings in women with a low level of fitness, its associated loss of ROM and strength, and a less healthy metabolic profile,²⁴, ⁴⁸, ⁴⁹ women cancer survivors may be at particular risk for early development of cardiac disease. The only way to identify at-risk patients is to perform a battery of assessments that measures impairments (ROM, strength, limb volume), performance (walk time, AMPS), and function (HAP, vocational and leisure activity).

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