Physical fitness in children with Marfan and Loeys-Dietz syndrome: associations between cardiovascular parameters, systemic manifestations, fatigue, and pain.

Children with Marfan (MFS) and Loeys-Dietz syndrome (LDS) report limitations in physical activities, sports, school, leisure, and work participation in daily life. This observational, cross-sectional, multicenter study explores associations between physical fitness and cardiovascular parameters, systemic manifestations, fatigue, and pain in children with MFS and LDS. Forty-two participants, aged 6-18 years (mean (SD) 11.5(3.7)), diagnosed with MFS (n = 36) or LDS (n = 6), were enrolled. Physical fitness was evaluated using the Fitkids Treadmill Test's time to exhaustion (TTE) outcome measure. Cardiovascular parameters (e.g., echocardiographic parameters, aortic surgery, cardiovascular medication) and systemic manifestations (systemic score of the revised Ghent criteria) were collected. Pain was obtained by visual analog scale. Fatigue was evaluated by PROMIS® Fatigue-10a-Pediatric-v2.0-short-form and PROMIS® Fatigue-10a-Parent-Proxy-v2.0-short-form. Multivariate linear regression analyses explored associations between physical fitness (dependent variable) and independent variables that emerged from the univariate linear regression analyses (criterion p < .05). The total group (MFS and LDS) and the MFS subgroup scored below norms on physical fitness TTE Z-score (mean (SD) -3.1 (2.9); -3.0 (3.0), respectively). Univariate analyses showed associations between TTE Z-score aortic surgery, fatigue, and pain (criterion p < .05). Multivariate analyses showed an association between physical fitness and pediatric self-reported fatigue that explained 48%; 49%, respectively, of TTE Z-score variance (F (1,18) = 18.6, p ≤ .001, r2 = .48; F (1,15) = 16,3, p = .01, r2 = .49, respectively).    Conclusions: Physical fitness is low in children with MFS or LDS and associated with self-reported fatigue. Our findings emphasize the potential of standardized and tailored exercise programs to improve physical fitness and reduce fatigue, ultimately enhancing the physical activity and sports, school, leisure, and work participation of children with MFS and LDS. What is Known: • Marfan and Loeys-Dietz syndrome are heritable connective tissue disorders and share cardiovascular and systemic manifestations. • Children with Marfan and Loeys-Dietz syndrome report increased levels of disability, fatigue and pain, as well as reduced levels of physical activity, overall health and health-related quality of life. What is New: • Physical fitness is low in children with Marfan and Loeys-Dietz syndrome and associated with self-reported fatigue. • Our findings emphasize the potential of standardized and tailored exercise programs to improve physical fitness and reduce fatigue, ultimately enhancing the physical activity and sports, school, leisure, and work participation of children with Marfan and Loeys-Dietz syndrome.

Metabolic load during morning care and active bed exercises in critically ill patients: An explorative study.

BACKGROUND: To avoid overexertion in critically ill patients, information on the physical demand, i.e., metabolic load, of daily care and active exercises is warranted. OBJECTIVE: The objective of this study was toassess the metabolic load during morning care activities and active bed exercises in mechanically ventilated critically ill patients. METHODS: This study incorporated an explorative observational study executed in a university hospital intensive care unit. Oxygen consumption (VO2) was measured in mechanically ventilated (≥48 h) critically ill patients during rest, routine morning care, and active bed exercises. We aimed to describe and compare VO2 in terms of absolute VO2 (mL) defined as the VO2 attributable to the activity and relative VO2 in mL per kilogram bodyweight, per minute (mL/kg/min). Additional outcomes achieved during the activity were perceived exertion, respiratory variables, and the highest VO2 values. Changes in VO2 and activity duration were tested using paired tests. RESULTS: Twenty-one patients were included with a mean (standard deviation) age of 59 y (12). Median (interquartile range [IQR]) durations of morning care and active bed exercises were 26 min (21-29) and 7 min (5-12), respectively. Absolute VO2 of morning care was significantly higher than that of active bed exercises (p = 0,009). Median (IQR) relative VO2 was 2.9 (2.6-3.8) mL/kg/min during rest; 3.1 (2.8-3.7) mL/kg/min during morning care; and 3.2 (2.7-4) mL/kg/min during active bed exercises. The highest VO2 value was 4.9 (4.2-5.7) mL/kg/min during morning care and 3.7 (3.2-5.3) mL/kg/min during active bed exercises. Median (IQR) perceived exertion on the 6-20 Borg scale was 12 (10.3-14.5) during morning care (n = 8) and 13.5 (11-15) during active bed exercises (n = 6). CONCLUSION: Absolute VO2 in mechanically ventilated patients may be higher during morning care than during active bed exercises due to the longer duration of the activity. Intensive care unit clinicians should be aware that daily-care activities may cause intervals of high metabolic load and high ratings of perceived exertion.

Somatic symptoms, pain, catastrophizing and the association with disability among children with heritable connective tissue disorders.

The aim of the present study was to investigate the nature and prevalence of nonspecific somatic symptoms, pain and catastrophizing in children with Heritable Connective Tissue Disorders (HCTD), and to determine their association with disability. This observational, multicenter study included 127 children, aged 4-18 years, with Marfan syndrome (MFS) (59%), Loeys-Dietz syndrome (LDS) (8%), Ehlers-Danlos syndromes (EDS) (12%) and hypermobile Ehlers-Danlos syndrome (hEDS) (23%). The assessments included the Children's Somatization Inventory or parent proxy (CSI, PCSI), pain visual-analogue scale (VAS), SUPERKIDZ body diagram, Pain Catastrophizing Scale Child or parent proxy (PCS-C, PCS-P) and Childhood Health Assessment Questionnaire (CHAQ-30). Data from children aged ≥8 years were compared to normative data. In children ≥ 8 years (n = 90), pain was present in 59%, with a median of 4 (IQR = 3-9) pain areas. Compared to normative data, the HCTD group reported significantly higher on the CSI (p ≤ 0.001, d = 0.85), VAS pain intensity (p ≤ 0.001, d = 1.22) and CHAQ-30 (p ≤ 0.001, d = 1.16) and lower on the PCS-C (p = 0.017, d = -0.82) and PCS-P (p ≤ 0.001, d = -0.49). The intensity of nonspecific somatic symptoms and pain explained 45% of the variance in disability (r2 = 0.45 F(2,48) = 19.70, p ≤ 0.001). In children ≤ 7 years (n = 37), pain was present in 35% with a median of 5(IQR = 1-13) pain areas. The mean(SD) VAS scores for pain intensity was 1.5(2.9). Functional disability was moderately correlated to the number of pain areas (r = 0.56, p ≤ 0.001), intensity of nonspecific somatic symptoms (r = 0.63, p ≤ 0.001) and pain (r = 0.83, p ≤ 0.001). In conclusion, this study supports the need for comprehensive assessment of nonspecific somatic symptoms, pain, and disability in children with HCTD to allow tailored treatment.

Development and evaluation of a fidelity tool in a post-discharge responsive parenting intervention program for very preterm born children.

The TOP program is a fully implemented responsive parenting intervention for very preterm born infants. Fidelity monitoring of interventions is important for preserving program adherence, impact outcomes and to make evidence-based adaptations. The aim of this study was to develop a fidelity tool for the TOP program following an iterative and co-creative process and subsequently evaluate the reliability of the tool. Three consecutive phases were carried out. Phase I: Initial development and pilot testing two methods namely self-report and video based observation. Phase II: Adaptations and refinements. Phase III: Evaluation of the psychometric properties of the tool based on 20 intervention videos rated by three experts.The interrater reliability of the adherence and competence subscales was good (ICC.81 to .84) and varied from moderate to excellent for specific items (ICC between .51 and .98). The FITT displayed a high correlation (Spearman's rho.79 to.82) between the subscales and total impression item. The co-creative and iterative process resulted in a clinical useful and reliable tool for evaluating fidelity in the TOP program. This study offers insights in the practical steps in the development of a fidelity assessment tool which can be used by other intervention developers.

Recommendations for an exercise intervention and core outcome set for older patients after hospital discharge: Results of an international Delphi study.

For older adults, acute hospitalization is a high-risk event with poor health outcomes, including functional decline. In absence of practical guidelines and high quality randomized controlled trials, this Delphi study was conducted. The aim of this study was to obtain consensus on an exercise intervention program, a core outcome set (COS) and handover information to prevent functional decline or restore physical function in acutely hospitalized older patients transitioning from hospital to home. An internal panel of experts in the field of exercise interventions for acutely hospitalized older adults were invited to join the study. In the Delphi study, relevant topics were recognized, statements were formulated and ranked on a 9-point Likert scale in two additional rounds. To reaching consensus, a score of 7-9 was classified as essential. Results were expressed as median and semi-interquartile range (SIQR), and consensus threshold was set at SIQR≤0.5. Fifteen international experts from eight countries participated in the panel. The response rate was 93%, 93% and 80% for the three rounds respectively. After three rounds, consensus was reached on 167 of the 185 (90.3%) statements, of which ninety-five (51.4%) were ranked as essential (median Likert-score ≥7.0, SIQR ≤0.5). This Delphi study provides starting points for developing an exercise intervention, a COS and handover information. The results of this Delphi study can assist physical therapists to provide a tailored exercise intervention for older patients with complex care needs after hospital discharge, to prevent functional decline and/or restore physical function.

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.

The additional value of e-Health for patients with a temporomandibular disorder: a mixed methods study on the perspectives of orofacial physical therapists and patients.

PURPOSE: To assess the experience and perceived added value of an e-Health application during the physical therapy treatment of patients with temporomandibular disorders (TMD). MATERIALS AND METHODS: A mixed-methods study including semi-structured interviews was performed with orofacial physical therapists (OPTs) and with TMD patients regarding their experience using an e-Health application, Physitrack. The modified telemedicine satisfaction and usefulness questionnaire and pain intensity score before and after treatment were collected from the patients. RESULTS: Ten OPTs, of which nine actively used Physitrack, described that the e-Health application can help to provide personalised care to patients with TMD, due to the satisfying content, user-friendliness, accessibility, efficiency, and ability to motivate patients. Ten patients, of which nine ended up using Physitrack, felt that shared decision-making was very important. These patients were positive towards the application as it was clear, convenient, and efficient, it helped with reassurance and adherence to the exercises and overall increased self-efficacy. This was mostly built on their experience with exercise videos, as this feature was most used. None of the OPTs or patients used all features of Physitrack. The overall satisfaction of Physitrack based on the telemedicine satisfaction and usefulness questionnaire (TSUQ) was 20.5 ± 4.0 and all patients (100%) showed a clinically relevant reduction of TMD pain (more than 2 points and minimally 30% difference). CONCLUSION: OPTs and patients with TMD shared the idea that exercise videos are of added value on top of usual physical therapy care for TMD complaints, which could be delivered through e-Health.Implications for RehabilitationPhysical therapists and patients with temporomandibular disorders do not use all features of the e-Health application Physitrack in a clinical setting.Exercise videos were the most often used feature and seen as most valuable by physical therapists and patients.Based on a small number of participants, e-Health applications such as Physitrack may be perceived as a valuable addition to the usual care, though this would need verification by a study designed to evaluate the therapeutic effect (e.g., a randomised clinical trial).

Bio-Electrical Impedance Analysis: A Valid Assessment Tool for Diagnosis of Low Appendicular Lean Mass in Older Adults?

Background: The diagnosis of sarcopenia is essential for early treatment of sarcopenia in older adults, for which assessment of appendicular lean mass (ALM) is needed. Multi-frequency bio-electrical impedance analysis (MF-BIA) may be a valid assessment tool to assess ALM in older adults, but the evidences are limited. Therefore, we validated the BIA to diagnose low ALM in older adults. Methods: ALM was assessed by a standing-posture 8 electrode MF-BIA (Tanita MC-780) in 202 community-dwelling older adults (age ≥ 55 years), and compared with dual-energy X-ray absorptiometry (DXA) (Hologic Inc., Marlborough, MA, United States; DXA). The validity for assessing the absolute values of ALM was evaluated by: (1) bias (mean difference), (2) percentage of accurate predictions (within 5% of DXA values), (3) the mean absolute error (MAE), and (4) limits of agreement (Bland-Altman analysis). The lowest quintile of ALM by DXA was used as proxy for low ALM (< 22.8 kg for men, < 16.1 kg for women). Sensitivity and specificity of diagnosing low ALM by BIA were assessed. Results: The mean age of the subjects was 72.1 ± 6.4 years, with a BMI of 25.4 ± 3.6 kg/m2, and 71% were women. BIA slightly underestimated ALM compared to DXA with a mean bias of -0.6 ± 1.2 kg. The percentage of accurate predictions was 54% with a MAE of 1.1 kg, and limits of agreement were -3.0 to + 1.8 kg. The sensitivity for ALM was 80%, indicating that 80% of subjects who were diagnosed as low ALM according to DXA were also diagnosed low ALM by BIA. The specificity was 90%, indicating that 90% of subjects who were diagnosed as normal ALM by DXA were also diagnosed as normal ALM by the BIA. Conclusion: This comparison showed a poor validity of MF-BIA to assess the absolute values of ALM, but a reasonable sensitivity and specificity to recognize the community-dwelling older adults with the lowest muscle mass.

Heritable connective tissue disorders in childhood: Decreased health-related quality of life and mental health.

The psychosocial consequences of growing up with Heritable Connective Tissue Disorders (HCTD) are largely unknown. We aimed to assess Health-Related Quality of Life (HRQoL) and mental health of children and adolescents with HCTD. This observational multicenter study included 126 children, aged 4-18 years, with Marfan syndrome (MFS, n = 74), Loeys-Dietz syndrome (n = 8), molecular confirmed Ehlers-Danlos syndromes (n = 15), and hypermobile Ehlers-Danlos syndrome (hEDS, n = 29). HRQoL and mental health were assessed through the parent and child-reported Child Health Questionnaires (CHQ-PF50 and CHQ-CF45, respectively) and the parent-reported Strengths and Difficulties Questionnaire. Compared with a representative general population sample, parent-reported HRQoL of the HCTD-group showed significantly decreased Physical sum scores (p < 0.001, d = 0.9) and Psychosocial sum scores (p = 0.024, d = 0.2), indicating decreased HRQoL. Similar findings were obtained for child-reported HRQoL. The parent-reported mental health of the HCTD-group showed significantly increased Total difficulties sum scores (p = 0.01, d = 0.3), indicating decreased mental health. While the male and female MFS- and hEDS-subgroups both reported decreased HRQoL, only the hEDS-subgroup reported decreased mental health. In conclusion, children and adolescents with HCTD report decreased HRQoL and mental health, with most adverse outcomes reported in children with hEDS and least in those with MFS. These findings call for systematic monitoring and tailored interventions.

Steps to recovery: Body weight-supported treadmill training for critically ill patients: A randomized controlled trial.

PURPOSE: Early mobilization of critically ill patients improves functional recovery, but is often hampered by tubes, drains, monitoring devices and muscular weakness. A mobile treadmill with bodyweight support facilitates early mobilization and may shorten recovery time to independent ambulation as compared to usual care physiotherapy alone. MATERIALS AND METHODS: Single center RCT, comparing daily bodyweight supported treadmill training (BWSTT) with usual care physiotherapy, in patients who had been or were mechanically ventilated (≥48 h) with ≥MRC grade 2 quadriceps muscle strength. BWSTT consisted of daily treadmill training in addition to usual care physiotherapy (PT). Primary outcome was time to independent ambulation measured in days, using the Functional Ambulation Categories (FAC-score: 3). Secondary outcomes included hospital length of stay and serious adverse events. RESULTS: The median (IQR) time to independent ambulation was 6 (3 to 9) days in the BWSTT group (n = 19) compared to 11 (7 to 23) days in the usual care group (n = 21, p = 0.063). Hospital length of stay was significantly different in favour of the BWSTT group (p = 0.037). No serious adverse events occurred. INTERPRETATION: BWSTT seems a promising intervention to enhance recovery of ambulation and shorten hospital length of stay of ICU patients, justifying a sufficiently powered multicenter RCT. TRIAL REGISTRATION NUMBER: Dutch Trial Register ID: NTR6943.

Using e-Health in the physical therapeutic care process for patients with temporomandibular disorders: a qualitative study on the perspective of physical therapists and patients.

BACKGROUND: Treatment of temporomandibular disorder (TMD) currently consists of a combination of noninvasive therapies and may be supported by e-Health. It is, however, unclear if physical therapists and patients are positive towards the use of e-Health. PURPOSE: To assess the needs, facilitators and barriers of the use of an e-Health application from the perspective of both orofacial physical therapists and patients with TMD. METHODS: A descriptive qualitative study was performed. Eleven physical therapists and nine patients with TMD were interviewed using a topic guide. Thematic analysis was applied, and findings were ordered according to four themes: acceptance of e-Health, expected utility, usability and convenience. RESULTS: Physical therapists identified the need for e-Health as a supporting application to send questionnaires, animated exercises and evaluation tools. Key facilitators for both physical therapists and patients for implementing e-Health included the increase in self-efficacy, support of data collection and personalization of the application. Key barriers are the increase of screen time, the loss of personal contact, not up-to-date information and poor design of the application. CONCLUSIONS: Physical therapists and patients with TMD are positive towards the use of e-Health, in a blended form with the usual rehabilitation care process for TMD complaints.Implications for rehabilitationThe rehabilitation process of temporomandibular complaints may be supported by the use of e-Health applications.Physical therapists and patients with temporomandibular disorders are positive towards the use of e-Health as an addition to the usual care.Especially during the treatment process, there is a need for clear animated videos and reminders for the patients.

Barriers to and solutions for improving physical activity in adults during hospital stay: a mixed-methods study among healthcare professionals.

PURPOSE: To identify healthcare professionals' perspectives on key barriers to improving physical activity in hospitalized adult patients, and to identify solutions to overcome these barriers. METHODS: We used an explanatory sequential mixed-methods study design in a Dutch university hospital. A survey exploring 39 potential barriers was completed by 15 physicians/physician assistants, 106 nurses, four nursing assistants, and four physical therapists working on surgery, internal medicine, and cardiology wards. Next, three in-depth semi-structured focus groups - comprising 30 healthcare professionals - discussed the survey findings to identify key barriers and solutions. Focus group discussions were analyzed using thematic analysis. RESULTS: Five themes were identified that described both the key barriers and the solutions to overcome these barriers. Healthcare professionals proposed several solutions, including clarifying the definition of physical activity, empowering patients to take responsibility for physical activity, giving physical therapists or physicians a prominent role in encouraging physical activity, and changing the hospital ward to entice patients to become physically active. CONCLUSIONS: Healthcare professionals need clear guidelines, roles, and responsibilities when it comes to physical activity. They also need personalized interventions that empower patients in physical activity. Finally, hospital wards should be designed and furnished so that patients are encouraged to be active.IMPLICATIONS FOR REHABILITATIONMany healthcare professionals want to sustainably improve physical activity in hospitalized adults.For this they need clear guidelines that not only define physical activity, but also describe the roles and responsibilities of all members of the medical team.Healthcare professionals need interventions that help to empower patients to take an active role in physical activity during hospital stay.Hospital wards should be designed and furnished so that patients are encouraged to be physically active.

Physical dependence and urinary catheters both strongly relate to physical inactivity in adults during hospital stay: a cross-sectional, observational study.

PURPOSE: To determine which factors are associated with physical inactivity in hospitalized adults of all ages. METHODS: A cross-sectional sample of 114 adults admitted to a gastrointestinal surgery, internal medicine or cardiology hospital ward (median age 60, length of stay 13 days) were observed during one random day from 8 am to 8 pm using wireless accelerometers and behavioral mapping protocols. Factors (e.g., comorbidities, self-efficacy, independence in mobility, functional restraints) were collected from medical records, surveys, and observations. RESULTS: Patients were physically active for median(IQR) 26 (13-52.3) min and were observed to lie in bed for 67.3%, sit for 25.2%, stand for 2.5%, and walk for 5.0% of the time. Multivariable regression analysis revealed that physical inactivity was 159.87% (CI = 89.84; 255.73) higher in patients dependent in basic mobility, and 58.88% (CI = 10.08; 129.33) higher in patients with a urinary catheter (adjusted R2 = 0.52). The fit of our multivariable regression analysis did not improve after adding hospital ward to the analysis (p > 0.05). CONCLUSIONS: Independence in mobility and urine catheter presence are two important factors associated with physical inactivity in hospitalized adults of all ages, and these associations do not differ between hospital wards. Routine assessments of both factors may therefore help to identify physically inactive patients throughout the hospital.IMPLICATIONS FOR REHABILITATIONHealthcare professionals should be aware that physical inactivity during hospital stay may result into functional decline.Regardless of which hospital ward patients are admitted to, once patients require assistance in basic mobility or have a urinary catheter they are at risk of physical inactivity during hospital stay.Implementing routine assessments on the independence of basic mobility and urine catheter presence may therefore assist healthcare professionals in identifying physically inactive patients before they experience functional decline.

Physical rehabilitation interventions in children with acquired brain injury: a scoping review.

AIM: To synthesize the evidence about the characteristics (frequency, intensity, time, type) and effects of physical rehabilitation interventions on functional recovery and performance in daily functioning in children and young people with acquired brain injury (ABI), including traumatic brain injuries (TBI) and non-TBI, during the subacute rehabilitation phase. METHOD: Using scoping review methodology, a systematic literature search was performed using four databases. Articles were screened by title and abstract and data from eligible studies were extracted for synthesis. RESULTS: Nine of 3009 studies were included. The results demonstrated a variety of intervention characteristics: frequency varied between 1 and 7 days per week; time of intervention varied between 25 minutes and 6 hours a day; intervention types were specified in seven studies; and none of the included studies reported details of intensity of intervention. All studies reported positive results on the International Classification of Functioning, Disability and Health: Children and Youth (ICF-CY) levels of body function and activities after the intervention period, with study designs of included studies being cohort studies without concurrent controls (n=7) or case reports (n=2). INTERPRETATION: Inconsistency in results hampers generalizability to guide clinical practice. Physical interventions during subacute rehabilitation have potential to improve functional recovery with intervention characteristics as an important factor influencing its effectiveness. Future well-designed studies are indicated to gain knowledge and optimize rehabilitation practice in paediatric ABI and high-quality research including outcomes across all ICF-CY domains is needed.

Feasibility of a home-based interdisciplinary rehabilitation program for patients with Post-Intensive Care Syndrome: the REACH study.

BACKGROUND: Survivors of critical illness experience long-term functional challenges, which are complex, heterogeneous, and multifactorial in nature. Although the importance of rehabilitation interventions after intensive care unit (ICU) discharge is universally recognized, evidence on feasibility and effectiveness of home-based rehabilitation programs is scarce and ambiguous. This study investigates the feasibility of an interdisciplinary rehabilitation program designed for patients with Post-Intensive Care Syndrome (PICS) who are discharged home. METHODS: A mixed method, non-randomized, prospective pilot feasibility study was performed with a 6-month follow-up, comparing the intervention (REACH) with usual care. REACH was provided by trained professionals and included a patient-centered, interdisciplinary approach starting directly after hospital discharge. Primary outcomes were patient safety, satisfaction, adherence, referral need and health care usage. Secondary outcomes, measured at 3 timepoints, were functional exercise capacity, self-perceived health status, health-related quality of life (HRQoL), return to work and psychotrauma. Risk of undernutrition was assessed at baseline. RESULTS: 43 patients with a median mechanical ventilation duration of 8 (IQR:10) days, were included in the study and 79.1% completed 6-month follow-up. 19 patients received the intervention, 23 received usual care. Groups were similar for gender distribution and ICU length of stay. No adverse events occurred. REACH participants showed higher satisfaction with treatment and reported more allied health professional visits, while the usual care group reported more visits to medical specialists. Qualitative analysis identified positive experiences among REACH-professionals related to providing state-of-the-art interventions and sharing knowledge and expertise within an interprofessional network. Similar recovery was seen between groups on all secondary outcomes, but neither group reached reference values for HRQoL at 6 months. Larger return to work rates were seen in the REACH group. Prevalence of undernutrition at hospital discharge was high in both groups (> 80%), warranting the need for careful tuning of physical therapy and nutritional interventions. CONCLUSIONS: This study shows that providing early, home-based rehabilitation interventions for patients with PICS-related symptoms is feasible and perceived positively by patients and professionals. When provided in an interdisciplinary collaborative network state of the art, person-centered interventions can be tailored to individual needs potentially increasing patient satisfaction, adherence, and efficacy. Registered in the Dutch Trial register: NL7792: https://www.trialregister.nl/trial/7792 , registered 7-06-2019.

Heritable Connective Tissue Disorders in Childhood: Increased Fatigue, Pain, Disability and Decreased General Health.

Heritable Connective Tissue Disorders (HCTD) show an overlap in the physical features that can evolve in childhood. It is unclear to what extent children with HCTD experience burden of disease. This study aims to quantify fatigue, pain, disability and general health with standardized validated questionnaires. METHODS: This observational, multicenter study included 107 children, aged 4-18 years, with Marfan syndrome (MFS), 58%; Loeys-Dietz syndrome (LDS), 7%; Ehlers-Danlos syndromes (EDS), 8%; and hypermobile Ehlers-Danlos syndrome (hEDS), 27%. The assessments included PROMIS Fatigue Parent-Proxy and Pediatric self-report, pain and general health Visual-Analogue-Scales (VAS) and a Childhood Health Assessment Questionnaire (CHAQ). RESULTS: Compared to normative data, the total HCTD-group showed significantly higher parent-rated fatigue T-scores (M = 53 (SD = 12), p = 0.004, d = 0.3), pain VAS scores (M = 2.8 (SD = 3.1), p < 0.001, d = 1.27), general health VAS scores (M = 2.5 (SD = 1.8), p < 0.001, d = 2.04) and CHAQ disability index scores (M = 0.9 (SD = 0.7), p < 0.001, d = 1.23). HCTD-subgroups showed similar results. The most adverse sequels were reported in children with hEDS, whereas the least were reported in those with MFS. Disability showed significant relationships with fatigue (p < 0.001, rs = 0.68), pain (p < 0.001, rs = 0.64) and general health (p < 0.001, rs = 0.59). CONCLUSIONS: Compared to normative data, children and adolescents with HCTD reported increased fatigue, pain, disability and decreased general health, with most differences translating into very large-sized effects. This new knowledge calls for systematic monitoring with standardized validated questionnaires, physical assessments and tailored interventions in clinical care.

Physical Functioning After Admission to the PICU: A Scoping Review.

OBJECTIVES: To conduct a scoping review to 1) describe findings and determinants of physical functioning in children during and/or after PICU stay, 2) identify which domains of physical functioning are measured, 3) and synthesize the clinical and research knowledge gaps. DATA SOURCES: A systematic search was conducted in PubMed, Embase, Cumulative Index to Nursing and Allied Health Literature, and Cochrane Library databases following the Preferred Reporting Items for Systematic Reviews and Meta-analyses extension for Scoping Reviews guidelines. STUDY SELECTION: Two investigators independently screened and included studies against predetermined criteria. DATA EXTRACTION: One investigator extracted data with review by a second investigator. A narrative analyses approach was used. DATA SYNTHESIS: A total of 2,610 articles were identified, leaving 68 studies for inclusion. Post-PICU/hospital discharge scores show that PICU survivors report difficulties in physical functioning during and years after PICU stay. Although sustained improvements in the long-term have been reported, most of the reported levels were lower compared with the reference and baseline values. Decreased physical functioning was associated with longer hospital stay and presence of comorbidities. A diversity of instruments was used in which mobility and self-care were mostly addressed. CONCLUSIONS: The results show that children perceive moderate to severe difficulties in physical functioning during and years after PICU stay. Longitudinal assessments during and after PICU stay should be incorporated, especially for children with a higher risk for poor functional outcomes. There is need for consensus on the most suitable methods to assess physical functioning in children admitted to the PICU.

Digitally Supported Dietary Protein Counseling Changes Dietary Protein Intake, Sources and Distribution in Community-Dwelling Older Adults.

Digitally supported dietary counselling may be helpful in increasing the protein intake in combined exercise and nutritional interventions in community-dwelling older adults. To study the effect of this approach, 212 older adults (72.2 ± 6.3 years) were randomised in three groups: control, exercise, or exercise plus dietary counselling. The dietary counselling during the 6-month intervention was a blended approach of face-to-face contacts and videoconferencing, and it was discontinued for a 6-month follow-up. Dietary protein intake, sources, product groups, resulting amino acid intake, and intake per eating occasion were assessed by a 3-day dietary record. The dietary counselling group was able to increase the protein intake by 32% at 6 months, and the intake remained 16% increased at 12 months. Protein intake mainly consisted of animal protein sources: dairy products, followed by fish and meat. This resulted in significantly more intake of essential amino acids, including leucine. The protein intake was distributed evenly over the day, resulting in more meals that reached the protein and leucine targets. Digitally supported dietary counselling was effective in increasing protein intake both per meal and per day in a lifestyle intervention in community-dwelling older adults. This was predominantly achieved by consuming more animal protein sources, particularly dairy products, and especially during breakfast and lunch.