Effects of specialist care lower limb orthoses on personal goal attainment and walking ability in adults with neuromuscular disorders.

BACKGROUND: Lower limb orthoses intend to improve walking in adults with neuromuscular disorders (NMD). Yet, reported group effects of lower limb orthoses on treatment outcomes have generally been small and heterogeneous. We propose that guideline-based orthotic care within a multidisciplinary expert setting may improve treatment outcomes. AIM: To examine the effectiveness of specialist care orthoses compared to usual care orthoses on personal goal attainment and walking ability. DESIGN: Cohort study. POPULATION: Adults with NMD who experienced walking problems due to calf and/or quadriceps muscle weakness and were provided with a specialist care lower limb orthosis between October 2011 and January 2021. METHODS: Three months after provision, the specialist care orthosis was compared to the usual care orthosis worn at baseline in terms of personal goal attainment (Goal Attainment Scaling (GAS)), comfortable walking speed (m/s), net energy cost (J/kg/m) (both assessed during a 6-minute walk test), perceived walking ability and satisfaction. RESULTS: Sixty-four adults with NMD were eligible for analysis. The specialist care orthoses comprised 19 dorsiflexion-restricting ankle-foot orthoses (AFOs), 22 stance-control knee-ankle-foot orthoses (KAFOs) and 23 locked KAFOs. Overall, 61% of subjects showed a clinically relevant improvement in GAS score. Perceived safety, stability, intensity, fear of falling and satisfaction while walking all improved (p≤0.002), and subjects were satisfied with their specialist care orthosis and the services provided. Although no effects on walking speed or net energy cost were found in combined orthosis groups, specialist care AFOs significantly reduced net energy cost (by 9.5%) compared to usual care orthoses (from mean (SD) 3.81 (0.97) to 3.45 (0.80) J/kg/m, p = 0.004). CONCLUSION: Guideline-based orthotic care within a multidisciplinary expertise setting could improve treatment outcomes in adults with NMD compared to usual orthotic care by improvements in goal attainment and walking ability. A randomized controlled trial is now warranted to confirm these results.

An exploratory study of clinical characteristics and gait features of adolescents with hypermobility disorders.

BACKGROUND: In adolescents with non-pathological and pathological joint hypermobility, gait deviations have been associated with pain and fatigue. It remains unclear what distinguishes the non-pathological form of joint hypermobility (JH) from pathological forms (i.e. hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorders (HSD). Our objective was to identify discriminative clinical characteristics and biomechanical gait features between adolescents with hEDS/HSD, JH, and healthy controls (HC). METHODS: Thirty-two adolescents were classified into three subgroups (hEDS/HSD=12, JH=5, HC=15). Clinical characteristics (e.g. pain intensity and surface, fatigue, functional disability) were inventoried. The gait pattern was assessed using a three-dimensional, eight-camera VICON MX1.3 motion capture system, operating at a sample rate of 100 Hz (VICON, Oxford, UK). Spatiotemporal parameters, joint angles (sagittal plane), joint work, joint impulse, ground reaction force and gait variability expressed as percentage using Principal Component Analysis (PCA) were assessed and analysed using multivariate analysis. Multivariate analysis data is expressed in mean differences(MD), standard error(SE) and P-values. RESULTS: The hEDS/HSD-group had significantly higher fatigue score (+51.5 points, p = <0.001) and functional disability (+1.6, p < .001) than the HC-group. Pain intensity was significantly higher in the hEDS/HSD-group than the other subgroups (JH; +37 mm p = .004, HC; +38 mm, p = .001). The hEDS/HSD-group showed significantly more gait variability (JH; +7.2(2.0)% p = .003, HC; + 7.8(1.4)%, p = <0.001) and lower joint work (JH; -0.07(0.03)J/kg, p = .007, HC; - 0.06(0.03)J/kg, p = .013) than the other subgroups. The JH-group showed significantly increased ankle dorsiflexion during terminal stance (+5.0(1.5)degree, p = .001) compared to hEDS/HSD-group and knee flexion during loading response compared to HC-group (+5.7(1.8) degree, p = .011). SIGNIFICANCE: A distinctive difference in gait pattern between adolescents with non-pathological and pathological joint hypermobility is found in gait variability, rather than in the biomechanical features of gait. This suggests that a specific gait variability metric is more appropriate than biomechanical individual joint patterns for assessing gait in adolescents with hEDS/HSD.

Use and Usability Of Custom-Made Knee-Ankle-Foot Orthoses In Polio Survivors with Knee Instability: A Cross-Sectional Survey.

OBJECTIVE: To investigate the use of custom-made knee-ankle-foot orthoses in daily life and differences in usability factors of knee-ankle-foot orthoses between users and discontinued users. DESIGN: Cross-sectional survey study. SUBJECTS: A total of 163 polio survivors provided with a knee-ankle-foot orthosis at an outpatient clinic of a university hospital. METHODS: Use and usability of knee-ankle-foot orthoses in daily life were assessed with a postal questionnaire. Usability factors were formulated using the International Organization for Standardization (ISO) 9241-11 standard. RESULTS: A total of 106 respondents (65%) returned the questionnaire. Of these, 98 were eligible for analysis. Seventy-four respondents (76%) reported using their knee-ankle-foot orthosis. Compared with discontinued users (24%), users experienced more limitations when walking without an orthosis (p = 0.001), were more often experienced with wearing a previous orthosis (p < 0.001) and were more often prescribed with a locked rather than a stance-control knee-ankle-foot orthosis (p = 0.015). Furthermore, users reported better effectiveness of their knee-ankle-foot orthosis (p < 0.001), more satisfaction with goals of use and knee-ankle-foot orthosis-related aspects (p < 0.001). CONCLUSION: The majority of polio survivors used their custom-made knee-ankle-foot orthoses in daily life. Factors related to continued use, such as walking ability without orthosis, expectations of the orthosis, previous orthosis experience and type of knee-ankle-foot orthosis provided, should be considered and discussed when prescribing a knee-ankle-foot orthosis in polio survivors.

Self-reported functional ambulation is related to physical mobility status in polio survivors; a cross-sectional observational study.

BACKGROUND: The condensed 3-level version of the self-reported ambulation classification by Perry is a validated, simple-to-use instrument in clinical practice to classify functional ambulation. OBJECTIVE: To further validate the clinical meaning of the classification for polio survivors, we compared physical mobility status across 3 functional ambulation categories and investigated the relation between physical mobility and functional ambulation category. METHODS: We investigated a convenience sample of 140 individuals with polio [mean (SD) age 59.4 (12.1) years; 74 men] who were able to walk at least indoors. For indicators of physical mobility status, we assessed the walked distance (m) and walking energy cost (Jkg-1m-1) during a 6-min walk test at a comfortable speed. Furthermore, self-reported physical functioning and fatigue were assessed with the 36-item Short Form Health Survey physical functioning scale (SF36-PF) and Fatigue Severity Scale (FSS), respectively. Self-reported functional ambulation was classified as household walker, limited community walker or full community walker. RESULTS: The mean (SD) walked distance, energy cost, and SF36-PF and FSS scores significantly differed between household walkers (n=48) and limited community walkers (n=63) [275 (67) m; 6.35 (1.80) Jkg-1m-1; 27.7 (13.5), 5.53 (1.06), respectively, and 323 (73) m; 5.49 (1.50) Jkg-1m-1; 40.1 (15.1); 4.81 (1.38) (P<0.018)] and full community walkers (n=29) [383 (66) m; 4.68 (0.85) Jkg-1m-1; 63.9 (18.5), 3.85 (1.54) (P<0.001)], with significant differences also present between limited and full community walkers (P<0.05). Walked distance and SF36-PF score were significantly associated with functional ambulation level, determining 46% of the variance in ambulation level. CONCLUSION: The simple, self-reported classification of functional ambulation in 3 levels is clinically meaningful for polio survivors because it consistently corresponds to differences in objective and self-reported indicators of physical mobility and, as such, can be used to better manage rehabilitation treatment.

Use and usability of custom-made dorsiflexion-restricting ankle-foot orthoses for calf muscle weakness in polio survivors: a cross-sectional survey.

BACKGROUND: Dorsiflexion-restricting ankle-foot orthoses (DR-AFOs) are often prescribed in polio survivors with calf muscle weakness to reduce or solve gait problems. However, orthoses are sometimes not being used and/or users are dissatisfied with the usability. AIM: To compare the usability of custom-made DR-AFOs provided in clinical care between users and discontinued users who have calf muscle weakness due to polio. DESIGN: Cross-sectional survey. SETTING: Outpatient post-polio university hospital clinic in the Netherlands. POPULATION: All polio survivors with calf muscle weakness, provided with a DR-AFO between 2004 and 2015 in our outpatient clinic. METHODS: DR-AFO use and usability according to the ISO 9241-11 standard were evaluated with a questionnaire sent out by postal mail. RESULTS: Forty of 57 questionnaires were returned. Five respondents did not fulfil the eligibility criteria. DR-AFO use among the 35 eligible respondents was 74%. Compared to discontinued users, users were significantly more often male (users: 16 of 26 vs. discontinued users: 0 of 9, P=0.001), more limited in their walking ability without DR-AFO (P=0.007), perceived more effectiveness, both overall (P=0.001) and on their personal goals of use (P=0.006), and were more satisfied with orthosis-related aspects (P=0.011), such as comfort. CONCLUSIONS: Almost three quarters of the polio survivors used their orthosis. Use was related to several aspects of usability, indicating that it is important to consider usability in the prescription process of DR-AFOs for polio survivors with calf muscle weakness. CLINICAL REHABILITATION IMPACT: When prescribing DR-AFOs, it is important to consider that the orthosis is most likely used when the experienced walking problems are large and the DR-AFO reduces these problems. We recommend discussing the patient's personal goals for DR-AFO use and the anticipated improvement on the individual's walking problems as well as possible hindrance of the orthosis during daily life activities. Furthermore, providers may need to pay extra attention to females and should particularly take care that the experienced fit and comfort are satisfactory. Incorporating these suggestions in clinical practice may further improve DR-AFO use among polio survivors with calf muscle weakness.

Stiffness modification of two ankle-foot orthosis types to optimize gait in individuals with non-spastic calf muscle weakness - a proof-of-concept study.

BACKGROUND: To reduce gait problems in individuals with non-spastic calf muscle weakness, spring-like ankle-foot orthoses (AFOs) are often applied, but they are not individually optimized to treatment outcome. The aim of this proof-of-concept study was to evaluate the effects of modifying the stiffness for two spring-like AFO types with shoes-only as reference on gait outcomes in three individuals with calf muscle weakness due to polio. METHODS: We assessed 3D gait biomechanics, walking speed and walking energy cost for shoes-only and five stiffness conditions of a dorsal-leaf-spring AFO and a spring-hinged AFO. Outcomes were compared between stiffness conditions in the two AFOs and three subjects. RESULTS: Maximum ankle dorsiflexion angle decreased with increasing stiffness in both AFOs (up to 6-8°) and all subjects. Maximum knee extension angle changed little between stiffness conditions, however different responses between the AFOs and subjects were observed compared to shoes-only. Walking speed remained unchanged across conditions. For walking energy cost, we found fairly large differences across stiffness conditions with both AFOs and between subjects (range 3-15%). CONCLUSIONS: Modifying AFO stiffness in individuals with non-spastic calf muscle weakness resulted in substantial differences in ankle biomechanics and walking energy cost with no effect on speed. Our results provide proof-of-concept that individually optimizing AFO stiffness can clinically beneficially improve gait performance.

Compensations in lower limb joint work during walking in response to unilateral calf muscle weakness.

BACKGROUND: Patients with calf muscle weakness due to neuromuscular disorders have a reduced ankle push-off work, which leads to increased energy dissipation at contralateral heel-strike. Consequently, compensatory positive work needs to be generated, which is mechanically less efficient. It is unknown whether neuromuscular disorder patients compensate with their ipsilateral hip and/or contralateral leg; and if such compensatory joint work is related to walking energy cost. RESEARCH QUESTION: Do patients with calf muscle weakness compensate for the increase in negative joint work by increasing positive ipsilateral hip work and/or positive contralateral leg work? And is the total mechanical work related with walking energy cost? METHODS: Seventeen patients with unilateral flaccid calf muscle weakness and 10 healthy individuals performed the following two tests: i) a barefoot 3D gait analysis at comfortable speed and matched control speed (i.e. 0.4 non-dimensional) to assess lower limb joint work and ii) a 6-minute walk test at comfortable speed to assess walking energy cost. RESULTS: Patients had a lower comfortable walking speed compared to healthy individuals (1.05 vs 1.36 m/s, p < 0.001) and did not increase positive lower limb joint work at comfortable speed. At matched speed (1.25 m/s), patients showed increased positive work at their ipsilateral hip (0.38 ± 0.08 vs 0.27 ± 0.07, p = 0.001) and/or contralateral leg (0.99 ± 0.14 vs 0.69 ± 0.14, p < 0.001). Patients with weakest plantar flexors used both strategies. No relation between total positive work and walking energy cost was found (r = 0.43, p = 0.122). SIGNIFICANCE: Patients with unilateral calf muscle weakness compensated for reduced ankle push-off work by lowering their comfortable walking speed or, at matched speed, by generating additional positive joint work at the ipsilateral hip and/or contralateral leg. The additional positive joint work at matched speed did not explain the elevated walking energy cost at comfortable speed, which needs further exploration.

Gait patterns in association with underlying impairments in polio survivors with calf muscle weakness.

The objective was to identify gait patterns in polio survivors with calf muscle weakness and associate them to underlying lower extremity impairments, which are expected to help in the search for an optimal orthosis. Unilaterally affected patients underwent barefoot 3D-gait analyses. Gait pattern clusters were created based on the ankle and knee angle and ankle moment shown in midstance of the affected limb. Impairment clusters were created based on plantarflexor and knee-extensor strength, and ankle and knee joint range-of-motion. The association between gait patterns and underlying impairments were examined descriptively. The Random Forest Algorithm and regression analyses were used to predict gait patterns and parameters. Seven gait patterns in 73 polio survivors were identified, with two dominant patterns: one with a mildly/non-deviant ankle angle, ankle moment and knee angle (n=23), and one with a strongly deviant ankle angle and a mildly/non-deviant ankle moment and knee angle (n=18). Gait pattern prediction from underlying impairments was 49% accurate with best prediction performance for the second dominant gait pattern (sensitivity 78% and positive predictive value 74%). The underlying impairments explained between 20 and 32% of the variance in individual gait parameters. Polio survivors with calf muscle weakness who present a similar impairment profile do not necessarily walk the same. From physical examination alone, the gait pattern nor the individual gait parameters could be accurately predicted. The patient's gait should therefore be measured to help in the prescription and evaluation of orthoses for these patients.

Ankle-foot orthoses that restrict dorsiflexion improve walking in polio survivors with calf muscle weakness.

In polio survivors with calf muscle weakness, dorsiflexion-restricting ankle-foot orthoses (DR-AFOs) aim to improve gait in order to reduce walking-related problems such as instability or increased energy cost. However, evidence on the efficacy of DR-AFOs in polio survivors is lacking. We investigated the effect of DR-AFOs on gait biomechanics, walking energy cost, speed, and perceived waking ability in this patient group. Sixteen polio survivors with calf muscle weakness underwent 3D-gait analyses to assess gait biomechanics when walking with a DR-AFOs and with shoes only. Ambulant registration of gas-exchange during a 6 min walk test determined walking energy cost, and comfortable gait speed was calculated from the walked distance during this test. Perceived walking ability was assessed using purposely-designed questionnaires. Compared with shoes-only, walking with the DR-AFOs significantly increased forward progression of the center of pressure (CoP) in mid-stance and it reduced ankle dorsiflexion and knee flexion in mid- and terminal stance (p < 0.05). Furthermore, walking energy cost was lower (-7%, p = 0.052) and gait speed was higher (p = 0.005). Patients were significantly more satisfied, felt safer, and less exhausted with the DR-AFO, compared to shoes-only (p < 0.05). DR-AFO effects varied largely across patients. Patients who walked with limited forward CoP progression and persisting knee extension during the shoes-only condition seemed to have benefitted least from the DR-AFO. In polio survivors with calf muscle weakness, DR-AFOs improved gait biomechanics, speed, and perceived walking ability, compared to shoes-only. Effects may depend on the shoes-only gait pattern, therefore further study is needed to determine which patients benefit most from the DR-AFO.

Reduced fat oxidation rates during submaximal exercise in adolescents with Crohn's disease.

BACKGROUND: Children with Crohn's disease (CD) suffer from malnutrition. Understanding substrate utilization during exercise may help patients with CD sustain a healthy active lifestyle without compromising nutrition. The aim of this study was to determine whether substrate utilization and bioavailability during exercise are altered in children with CD compared with controls. METHODS: Seven children with CD (mean age ± SD: 15.2 ± 2.3 yr) and 7 controls (14.4 ± 2.3 yr) were matched by sex and biological age. Participants completed 60 minutes of cycling at an intensity equivalent to 50% of their peak mechanical power. Rates of total fat and carbohydrate (CHO) oxidation, the amount of fat and CHO oxidized, and the contribution of fat and CHO to total energy expenditure were calculated from expired gases collected during exercise. Blood was collected before, during, and at the end of exercise and analyzed for insulin, free fatty acids, and glucose. RESULTS: Whole-body fat oxidation rate (expressed in mg · kg(-1) of body weight per min) during exercise was lower in children with CD (5.8 ± 1.0) compared with controls (8.0 ± 2.2, P < 0.05). Children with CD relied significantly more on CHO, with approximately 10% greater contribution toward total energy expenditure (P < 0.05) than controls. There were no differences in plasma insulin, free fatty acids, or glucose between the groups. CONCLUSIONS: Fat metabolism during exercise seems to be impaired in children with CD. A greater reliance on CHO is required to meet the energy demands of submaximal exercise.

Inflammatory and growth factor response to continuous and intermittent exercise in youth with cystic fibrosis.

BACKGROUND: Children with cystic fibrosis (CF) tend to suffer from chronic systemic inflammation and may have impaired growth associated with muscle catabolism. Therefore, investigating which type of exercise can elicit an anabolic response with minimal inflammation is of clinical value. METHODS: Twelve children with CF (mean±SD; age: 14.7±2.3 years, predicted FEV(1): 90.0±21.6%) and biological age-matched controls (age: 13.9±2.1 years) completed moderate-intensity, continuous exercise (MICE) and high-intensity, intermittent exercise (HIIE) on separate days. During each exercise, blood was drawn at various time points and analyzed for immune cells, inflammatory cytokines, and growth mediators. RESULTS: At rest, children with CF had higher concentrations of neutrophils and IL-6 compared with controls. In children with CF, HIIE did not affect immune cell subsets or cytokines: TNF-α, IL-6, and tumor necrosis factor-like weak inducer of apoptosis (TWEAK). All immune cell subsets and IL-6 increased significantly with MICE in both groups. Growth hormone (GH) increased with both types of exercise, with a greater change from rest during MICE. CONCLUSIONS: HIIE was a sufficient stimulus to increase GH in children with CF, without affecting systemic inflammation.

Exercise capacity in pediatric patients with inflammatory bowel disease.

OBJECTIVE: To examine exercise capacity in youth with Crohn's disease (CD) and ulcerative colitis (UC). STUDY DESIGN: Eleven males and eight females with CD and six males and four females with UC participated. Patients performed standard exercise tests to assess peak power (PP) and mean power (MP) and peak aerobic mechanical power (W(peak)) and peak oxygen uptake (VO(2peak)). Fitness variables were compared with reference data and also correlated with relevant clinical outcomes. RESULTS: Pediatric patients with inflammatory bowel disease had lower PP (∼90% of predicted), MP (∼88% of predicted), W(peak) (∼91% of predicted), and VO(2peak) (∼75% of predicted) compared with reference values. When patients with CD or UC were compared separately to reference values, W(peak) was significantly lower only in the CD group. No statistically significant correlations were found between any exercise variables and disease duration (r = 0.01 to 0.14, P = .47 to .95) or disease activity (r = -0.19 to -0.31, P = .11 to .38), measured by pediatric CD activity index or pediatric ulcerative colitis activity index. After controlling for chronological age, recent hemoglobin levels were significantly correlated with PP (r = 0.45, P = .049), MP (r = 0.63, P = .003), VO(2peak) (r = 0.62, P = .004), and W(peak) (r = 0.70, P = .001). CONCLUSIONS: Pediatric patients with inflammatory bowel disease exhibit impaired aerobic and anaerobic exercise capacity compared with reference values.

The effects of acute and chronic exercise on inflammatory markers in children and adults with a chronic inflammatory disease: a systematic review.

BACKGROUND: Chronic inflammatory diseases strike millions of people all over the world, and exercise is often prescribed for these patients to improve overall fitness and quality of life. In healthy individuals, acute and chronic exercise is known to alter inflammatory markers; however, less is known about these effects in patients with a chronic inflammatory disease. OBJECTIVE: The purpose of this review is to clearly define the effects of acute and chronic exercise on inflammatory markers in patients compared with healthy controls to determine whether exercise elicits an abnormal inflammatory response in those patients. DATA SOURCES: A literature search was conducted through MEDLINE and EMBASE (until January 2009). STUDY SELECTION: A distinction was made between children and adults, acute (i.e., single exercise session) and chronic exercise (i.e., training) and endurance and resistance exercise. To evaluate and compare the exercise responsiveness of various reported inflammatory markers, pre- to post-test effect sizes were calculated. DATA EXTRACTION: A methodological quality scoring as well as an assessment of the quality of exercise paradigms were both made. RESULTS: In total, 19 studies were included in this systematic review (children, n=7; adults, n=12). Of these, 7 were acute exercise studies in children, 8 were acute exercise in adults, 5 were chronic endurance exercise training studies, and I was a chronic resistance exercise training study. No exercise training studies were found involving children. Single bouts of exercise might elicit an aggravated inflammatory response in patients; this was reported for patients with type I diabetes mellitus, cystic fibrosis and chronic obstructive pulmonary disease. More severely affected patients may experience a more aggravated inflammatory response. Levels ofinflammatory markers, principally IL-6 but also T-cells, total leukocytes and lymphocytes, remained elevated longer into the recovery period following an acute bout of exercise in patients compared with healthy controls. Evidence was found that chronic endurance exercise training programs can attenuate systemic inflammation in patients with chronic heart failure and type 2 diabetes mellitus. CONCLUSIONS: In patients with a chronic inflammatory disease, both acute and chronic exercise might elicit different inflammatory responses (i.e., exaggerated after acute exercise & attenuated after training) compared to healthy matched controls. However, the results reveal a major gap in our knowledge regarding the effects of acute and chronic exercise on inflammatory markers in patients with a chronic inflammatory disease. Results are often inconsistent, and differences in training programs (intensity, frequency and duration), heterogeneity of disease populations studied, and analytic methods may be just some of the causes for these discrepancies. To optimize exercise prescriptions and recommendations for patients with a chronic inflammatory disease, more research is needed to define the nature of physical activity that confers health benefits without exacerbating underlying inflammatory stress associated with disease pathology.