Marchese, Victoria G. PT, PhD; Oriel, Kathryn N. PT, EdD; Fry, Jessica A. PT; Kovacs, Jamie L. PT; Weaver, Ryan L. PT; Reilly, Maureen M. RN, BSN; Ginsberg, Jill P. MD
Lower-extremity sarcomas of bone and soft tissue include Ewing's sarcoma, osteosarcoma, rhabdomyosarcoma, and synovial sarcoma. These tumors account for approximately 11% of all pediatric cancers.1 Therapy is multimodal and often includes aggressive chemotherapy in addition to surgery and/or radiation.1,2 Innovative surgical procedures, amputation/limb sparing/rotationplasty, and advances in chemotherapy and radiation have significantly contributed to improved outcomes.3–7 However, these procedures oftentimes still cause severe impairments in body structure/function, activity limitations, and participation restrictions.
The short- and long-term effects from the surgical procedures include limitations in range of motion, strength, pain, and endurance.8–17 Chemotherapy-related effects include vincristine peripheral neuropathy, corticosteroid-related osteonecrosis, and anthracycline-induced cardiovascular disease.10,12,13 Adverse effects of radiation include skin adhesions and fibrosis.12 These impairments in body structure/function may lead to activity limitations such as decreased ambulation speed and distance, difficulty ascending/descending stairs, and performing transitional movements such as sit to stand.8–14 Because of the improved survival rates in this pediatric population, we must focus on improving the functional mobility and quality of life of children, adolescents, and young adults with lower-extremity sarcoma. Thus, there is a need to identify tests that appropriately measure long-term impairments in body structure, activity limitations, and participation restrictions.11,12,18
The most commonly used outcome measure designed specifically for patients with lower-extremity tumors is the Musculoskeletal Tumor Society Rating Scale.19 This tool is primarily a physician-/nurse-administered assessment based on the patient report of pain, function, emotional acceptance, use of supports, walking, and gait. The Musculoskeletal Tumor Society Rating Scale is a subjective measure, dependent on the opinion of the person giving the ratings and contains no objective measures of functional mobility. Another commonly used tool is the Toronto Extremity Salvage Score (TESS) lower extremity version.20 The TESS is a self-administered questionnaire that allows participants to indicate the level of difficulty experienced in dressing, grooming, mobility, work, sports, and leisure. The TESS does not include any objective measures of functional mobility.
Physical therapists have a variety of norm and criterion-referenced tests to choose from when examining motor development, motor proficiency, and functional assessment in children, such as the Peabody Developmental Motor Scales, Bruininks-Oseretsky Test of Motor Proficiency, Gross Motor Function Measure, and Pediatric Evaluation of Disability Inventory. However, these outcome measures are oftentimes not appropriate for the needs of patients and survivors of lower-extremity sarcoma due to either the age limits of the scale or the actual complexity or simplicity of the items on the test. Physical therapists may also choose to select a battery of single-item tests that best fit the patients' specific needs versus 1 test with many items. Two examples of outcome measures are Timed Up and Go (TUG) and 9-minute run-walk. These 2 measures have reference data available; however, the TUG reference data are for an older population and a limited younger population, and the 9-minute run-walk reference values were obtained from performances on a large track (laps), which is not appropriate for clinical practice.21,22 The choice of test may depend on the clinician preference (physical therapist or physician) and may vary from practitioner to practitioner. To identify body function impairments, activity limitations, and participation restrictions in this specific patient population, an outcome measure that includes both subjective and objective measures of all components of the International Classification of Functioning, Disability, and Health is required.23
The Functional Mobility Assessment (FMA) was developed to fill the need of such a functional outcome measure for this specific patient population (Table 1).11,18 The FMA combines subjective and objective outcome measures. The FMA is a reliable and valid tool to measure functional mobility in childhood cancer survivors with lower-extremity sarcoma.16 Several motor tasks such as walking, running, and stair climbing are evaluated.11 The 6 subcategories of the FMA are (1) pain; (2) function with 2 specific measures, timed up and down stairs (TUDS) and TUG time, heart rate (HR), and rate of perceived exertion (RPE) measured during the TUDS and TUG; (3) supports; (4) satisfaction with walking quality; (5) participation in work, school, sports; and (6) endurance measured by the 9-minute run-walk test, HR and RPE are also measured during the 9-minute run-walk.11 The raw scores in each subcategory in the FMA are converted to a table score. The table scores are from 0 (worst) to 5 (best), with a maximum score of 70 points. The FMA allows the clinician to subjectively and objectively measure the patient's progress and focus on persistent body structure/function impairments (subcategories 1, 2, and 6), activity limitations (subcategories 2, 3, and 6), and participation restrictions (subcategories 4 and 5).16
The purpose of this study was to establish reference data from children, adolescents, and young adults who are healthy specifically for the FMA. Considering the well-known gender and age differences in motor skills and walking speed of children, adolescents, and young adults, our study provides gender- and age-specific reference values.22,24 Reference data on this new tool will allow health care professionals to make clinically relevant comparisons between the body structure/function, activity, and participation of healthy children and patients/survivors with lower-extremity sarcoma. In addition, the data may assist with providing justification for physical therapy services even when the child, adolescent, or young adult has completed medical treatment for cancer but continues to have body structure/function impairments, activity limitations, and participation restrictions as compared to age- and gender-matched peers. Thus, by having a standard outcome measure that is easy to administer, clinicians are more likely to make an appropriate referral to a physical therapist.
Participants included 503 healthy children and adolescents, aged 10 to 21 years, from Lebanon Valley College, Annville, Pennsylvania; Cedar Crest Middle School, Lebanon, Pennsylvania; Cedar Crest High School, Lebanon, Pennsylvania; and Julia R. Masterman Laboratory and Demonstration School, Philadelphia, Pennsylvania. To be eligible for this study, participants were required to meet several criteria, including (1) no known cardiorespiratory complications expected to affect physical function as reported by the participant or participants' caregiver; (2) no history of a neuromuscular disorder or lower-extremity abnormality, such as a fracture or amputation; and (3) no history of lower-extremity sarcoma.
The FMA is composed of 6 subcategories: (1) pain; (2) function with 2 specific measures, TUDS time and TUG time and both HR and RPE are also measured during the TUDS and TUG; (3) supports; (4) satisfaction with walking quality; (5) participation in work, school, sports; and (6) endurance as measured by the 9-minute run-walk test. Physiologic cost index (PCI), HR, and RPE are also measured during the 9-minute run-walk.
Raters performing the FMA with the participants included 5 pediatric physical therapists, 3 nurses, and 1 physician. To ensure consistency of data collection, each rater received training before performing the assessment with the participants. The training program was written in a log and provided to each rater. The training log included language that each therapist would use with the participants throughout the testing. For example, on the 9-minute run-walk, the raters could say to the participants “you are allowed to walk if you need to.” The first author provided the training for all the raters to allow for consistency of the instruction. During this training, each rater performed the scoring measures on 5 people while the first author observed the process. Formal intra- and interrater reliability testing was not performed for each rater because of the observed accuracy of each rater during the training session. In addition, intraclass correlation coefficients (ICCs) for intrarater reliability ICCs [3,1] and interrater reliability ICCs [2,1] ranging from 0.97 to 0.99 were achieved for the FMA with a mixed group of professionals on a prior study.16
This was a collaborative study between Lebanon Valley College and The Children's Hospital of Philadelphia. This study was approved by both Institutional Review Boards. Prior to students' participation in this study, the investigators of this study met with the principals of each of the schools and received school board approval to perform this study at each of the separate institutions. Following this approval, consent forms with an attached letter to parents were sent home with the students. The students who returned the consent forms were allowed to participate in the study. The investigators of this study worked closely with the physical education teachers at the middle and high schools to coordinate the data collection during the students' physical education class period. The college students who participated in this study were asked to participate by the investigators through verbal requests during class periods, sorority/fraternity meetings, and interactions with friends/family.
The examiners performed the data collection at 2 middle schools, 2 high schools, and 1 college. Participants performed the outcome measure tasks in a single period of time. The outcome measures were consistently gathered in the following order: resting HR, pain scale score, satisfaction with walking quality, supports, walking quality, participation, TUDS, TUG, and the 9-minute run/walk. During the TUDS, TUG, and 9-minute run/walk, participants' HR and RPE were obtained. In addition, PCI was calculated for the 9-minute run/walk.
The primary objective of this study was to develop normative values for the functional mobility subcategories of the FMA; therefore, descriptive statistics such as means, standard deviations, medians, and ranges (FMA total, pain, supports, satisfaction with my walking quality, and participation, TUDS time, TUDS HR, TUDS RPE, TUG time, TUG HR, TUG RPE, 9-minute run/walk distance, 9-minute run/walk HR, 9-minute run/walk RPE, and 9-minute run/walk PCI) were obtained in data analysis. The median and ranges were provided for the age categories for the males and females, so that the wide range in values could be appreciated. This is important for clinicians to note when making decisions regarding the need for physical therapy intervention for children with lower-extremity sarcoma.
The Mann-Whitney U test was performed to determine whether there was a significant difference between males and females on the FMA total score. The Kruskal-Wallis analysis of variance by ranks was used to identify whether there was a significant difference between age levels for both males and females.
Data were analyzed on 503 participants (260 females and 243 males) in this study. Table 2 provides the number of participants in each age group for males and females. Table 3 presents the frequency of participants across different races and gender. Races were represented similarly in all age groups except for the 18- to 21-year age group where there were no African American participants. The means and standard deviations for all participants were as follows: TUDS 6.18 ± 0.8 seconds, TUG 3.78 ± 0.6 seconds, 9-minute run/walk distance 4161 ± 893 feet, and FMA total 59 ± 3. Median and ranges were calculated for each age category with the females and males separated (Tables 4 and 5).
Table 4 and 5 do not include the FMA subcategory supports, satisfaction with my walking quality, or participation, because the maximum scores were achieved for all participants. Pain was also not included because on the 0 to 10 pain scale, 94% of the participants reported zero pain, 12 participants reported a 1, 4 participants reported a 2, 7 participants reported a 3, 2 participants reported a 4, and 1 participant reported a 7. The participants who reported experiencing pain stated that the pain would not limit them from participating in the study (pain in a finger, shoulder, skin, and back, headache, and menstrual discomfort). These subcategory scores were included in the data analyses for the FMA total scores.
None of the participants used any assistive devices (cane, walker, and crutches), thus reporting a score of 5 on the FMA. All participants reported that they were “very satisfied” with their walking quality, thus reporting a 5 on the FMA. Regarding participation, all participants reported either an FMA score of 5 “participating in work or school and sports” or an FMA score of 4 “participating in all activities including work/school, but limited in sports.”
The FMA outcome measure provides a possible maximum score of 70 when totaling the table scores in each subcategory; the median total FMA score for all the females was 58.81 (range, 46–68) and males 59.74 (range, 47–68). This healthy sample achieved scores greater than or equal to a 3 in all subcategories of the FMA.
A significant difference was found between females and males for total FMA score (P = .02) with males achieving higher scores than females. Significant differences were found between ages of both males and females for the FMA total score (P < .01).
The purpose of this study was to obtain reference values for individuals who are healthy performing the FMA to allow for the comparison of FMA scores of children, adolescents, and young adults with lower-extremity sarcoma to gender- and age-matched children, adolescents, and young adults who are healthy. The healthy sample scored at or more than a 3 in all subcategories of the FMA, thus supporting the recommendation that patients with lower-extremity sarcoma who perform less than a 3 on any subcategory would benefit from a physical therapy evaluation. The clinical relevance of this information is that any patient scoring less than a 3 in any subcategory or less than a 42 total FMA score warrants a more comprehensive examination by the clinician and/or a referral to physical therapy.
The TUG scores for our study were similar for those of other studies that examined a healthy population. Gocha-Marchese et al25 reported TUG scores, in seconds, as part of a study to examine strength and functional mobility in children with acute lymphoblastic leukemia as compared with age- and gender-matched reference values for children aged 4 to 15 years. The mean TUG scores, in seconds, for the children who were developing typically were 4.0 ± 0.81, whereas, for our study, the mean TUG score, in seconds, for children aged 10 to 14 years was 3.8 ± 0.64. Katz-Leurer et al26 reported as part of a study to examine balance abilities and gait characteristics, TUG time in seconds in a group of children who were developing typically with a mean age of 9 ± 2 years.26 The mean TUG times reported were 5.8 ± 0.6, whereas, for our study, the mean TUG time for our 10-year age group was lower with a mean time of 3.9 ± 0.7 seconds. In addition, Williams et al27 reported mean TUG time of 5.9 ± 1.3 seconds in 5-year-old children who were developing typically.27 In sharp contrast to these studies of children, the mean TUG times for the healthy older adult population are much higher. Thompson and Medley21 reported mean TUG times, 12.9 ± 1.3 seconds, for a 64- to 87-year-old healthy control group in a study on performance of individuals with Parkinson disease.
There are limited studies that have obtained reference values for the TUDS. However, Zaino et al,24 in their study of reliability and validity of the TUDS, reported median TUDS times for children who were developing typically (13 males and 14 females) for 3 separate age groups 8 to 10 years of age (n = 14), 11 to 12 years of age (n = 6), and 13 to 14 years of age (n = 7). Median TUDS times, in seconds, were as follows for the 3 age groups, 8.3 (7.4–12.6), 7.1 (6.3–10.3), and 7.5 (6.3–8.3), respectively, as compared with our study, in which median TUDS times, in seconds, for these same age groups were 6.2 (5.1–7.5), 5.9 (4.5–8.4), and 6.0 (3.8–8.1), respectively.
The Health Related Physical Fitness: Test Manual provides reference values for children and adolescents aged 5 to 18 years who are healthy for the 9-minute run-walk.22 The testing manual recommends running laps; however, this is not feasible in a hospital or outpatient hospital or clinic. Therefore, the 9-minute run-walk was modified by having participants run-walk 65-ft lengths (run out 65 ft, turn around, and return 65 ft), similar to what could be performed in a clinical setting. Therefore, when comparing the distance covered in the 9-minute time period, the type of course must be considered. The original 9-minute run-walk requires the participants to run in a circular type lap pattern where the FMA requires the participants to perform start and stop movements at each turn. Thus, it would be expected that the original 9-minute run-walk reference values would be higher than the FMA 9-minute run-walk reference values. This was consistent with our findings. For example, the 50 percentile score for both the original study and our study were found to be: 10-year-old males, 5070 ft versus 4154 ft; 10-year-old females, 4380 ft versus 3512 ft; 17-year-old males, 6507 ft versus 4675 ft; 17-year-old females, 5187 ft versus 4550 ft, respectively.
Regarding the pain and participation scores, it is the authors' recommendation that these numbers need to be examined in context for each individual on the day the FMA is administered. The individual may report pain, but the pain may be due to a headache that just occurred that day or pain in an extremity that has been present for a week.
The authors recognize the limitations of this study regarding the small sample size, specifically for the 10-year-old children. This study also has an imbalance of racial diversity in the 18- to 21-year age group, thus limiting the generalizability of the data. Also, this study does not report body mass index for the participants. With these limitations considered, the authors still support the use of the reference data of the FMA tool established in this study as a means to assist health care professionals (physical therapists, physicians, nurses) in making clinically relevant decisions regarding the functional abilities of children with lower-extremity sarcoma. These reference data may also help clinicians in guiding the focus of physical therapy services. In addition, the data may assist in providing justification (insurance companies, school-based physical therapy) for the need of physical therapy services after medical treatments are completed following an amputation, limb-sparing, or rotationplasty when the individual presents with secondary complications from the surgery and/or chemotherapy treatments, such as body function impairments (pain, decreased endurance, postural deformities), activity limitations (decreased speed and distance of walking, limitations in stair climbing), or participation restrictions (decreased participation in school sports activities, social functions, or employment).
In previous studies, intra- and interrater reliability has been excellent for the FMA, TUG, TUDS, and 9-minute run-walk with ICCs [3,1] and [2,1] ranging from 0.93 to 0.99.16,24,25 A weakness of our study might be that these measurements were not obtained on the specific raters in this study.
The FMA, with the 6 subcategories, was developed to measure functional mobility with a focus on all components of the International Classification of Functioning, Disability, and Health model in individuals who have survived lower-extremity sarcoma. To better serve the clinicians using the FMA, reference data were collected to allow health care professionals to compare the functional abilities of children, adolescents, and young adults with lower extremity sarcoma to age- and gender-matched children, adolescents, and young adults who are healthy. Future research should focus on the effectiveness of physical therapy interventions for patients with and survivors of lower-extremity sarcoma. In addition, the FMA could be used to monitor a patient's progress during a course of physical therapy.
The authors thank Jennifer Price, DPT, from Penn State Hershey Medical Center Children's Hospital, and the Director of Secondary Education and Physical Education teachers from Cornwall-Lebanon School District. The authors thank the Physical Education teachers from the Masterman School for their support.